Fluctuating Hearing Loss Research Articles

Bilateral Endolymphatic Hydrops in a Patient With Migraine Variant Without Vertigo: A Case Report

To use modern high-resolution inner ear imaging modalities to evaluate for endolymphatic hydrops (EH) in a patient with migraine-associated fluctuating hearing loss without vertigo spells or dizziness. EH has been well described in patients with Meniere's disease on both human temporal bone studies and modern high-resolution imaging; however, there is no study to date, to our knowledge, that examines the presence of EH in a patient with migraine and bilateral hearing loss. We present the MRI findings using a sequence for detecting EH in a unique case of a patient experiencing migraine headaches accompanied by fluctuating hearing loss without vertigo. Magnetic resonance imaging sequences included "cisternographic" three-dimensional T2, and delayed intravenous-enhanced three-dimensional fluid-attenuation inversion recovery (DIVE-3D-FLAIR) sequences, performed with 2350 ms (bright perilymph) and 2050 ms (bright endolymph) inversion times. The bright endolymph images were subtracted from bright perilymph images to create a composite image with bright perilymph, dark endolymph, and intermediate bone signals. A 40-year-old female presented with a left-sided sensorineural hearing loss and severe migraine headaches that began at age 12. For the past year, she experienced severe migraines with right-sided fluctuating sensorineural hearing loss, tinnitus, and aural fullness. Audiometry confirmed a drop of right-sided hearing at times of migraines and increased symptom severity. Vestibular testing was within normal limits. MRI demonstrated the presence of severe bilateral vestibular and cochlear EH. EH of both the cochlea and vestibule can be present in patients without Meniere's disease or vertigo. The relationship between migraine and Meniere's disease may be complex, as demonstrated in this patient with migraine-associated bilateral hearing loss with MRI documentation of severe bilateral EH. The fact that migraine can be associated with EH is important and demonstrates a potential relationship between the pathophysiology of migraine and that of EH. Given this patient's previous association of migraine and hearing loss at age 12, it appears that migrainous attacks occur simultaneously with the hearing loss, and may be potentially causative of the fluctuating hearing loss, mediated possibly through the development of EH. New imaging modalities allow for studies into the field of inner ear pathology, with significant implications for future research.

Histopathology of Meniere’s Disease

Meniere’s Disease is a pathologic condition of the inner ear characterized by the presence of sudden bouts of vertigo, fluctuating hearing loss, tinnitus, and/or aural fullness. Although much has been discovered on this disease since the first description by Prosper Meniere, the pathophysiology of the events leading to the clinical symptoms of the disease is still under debate. This study aims to perform an up-to-date review on Meniere’s Disease, focusing on histopathology, pathophysiology, genetics, and causative mechanisms.

Cochlear implantation in Meniere’s disease

Meniere’s disease (MD) is characterized by fluctuating hearing loss, episodic vertigo, tinnitus, and aural fullness. Whether secondary to intervention or to natural disease progression, a considerable subset of patients with MD has nonserviceable hearing in at least one ear. As such, cochlear implantation counseling and decision-making in the setting of MD must take into account the patient׳s surgical history. In patients who have undergone prior surgical labyrinthectomy, particular care must be taken in identifying the incus and facial nerve as the lateral semicircular canal has been removed. Labyrinthectomy and cochlear implantation can also be performed simultaneously, with most authors identifying and exposing the round window through a posterior tympanotomy before undertaking the labyrinthectomy. Although there were initial concerns regarding audiometric outcomes due to neural damage or ossification following implantation after a prior labyrinthectomy, studies have shown that the audiologic benefits are similar to the general postlingually deafened adult population.

Long-term measurements using home audiometry with Békésy’s technique

Objective: To examine the efficacy of fixed-frequency Békésy’s home audiometry to assess hearing fluctuation and treatment outcomes in patients with subjectively fluctuating hearing loss. Design: SMAPH, a software audiometry program for Windows, was installed and calibrated on laptop computers. Békésy’s audiometry was carried out daily in the patients’ homes, using sound-attenuating earphones. Study sample: Seventeen patients with previously or currently subjectively fluctuating hearing loss. Five patients received of treatment for their conditions during the measurement period. Results: Measurement periods ranged from 6 to 60 days. Varying degrees of compliance were seen, some patients measuring less than 50% of the days, others measuring every day. Based on their long-term measurements the patients were classified into three groups: patients with stable recordings, with fluctuating low-frequency hearing loss, or with fluctuating high-frequency hearing loss. In the patients with stable recordings, significant test–retest differences were seen below 10 dB at frequencies 0.125–8 kHz. Conclusions: Home audiometry with Békésy’s technique can be used to evaluate disease activity and to monitor hearing results after therapy.

Preceding profound deafness and co-factors promote development of endolymphatic hydrops in preliminary patients with delayed endolymphatic hydrops

Conclusion: Profound deafness (PD) is unlikely to be the only cause of endolymphatic hydrops (EH) in patients with delayed endolymphatic hydrops (DEH). Further studies in large patient cohorts are required to determine the etiologic co-factors involved in the development of EH in patients with PD and DEH.Objectives: DEH usually occurs in patients with sustained unilateral PD who then develop episodic vertigo or fluctuating hearing loss. The latency can vary markedly between PD and development of DEH, but the factors affecting inner ear homeostasis and causing EH during this latency period remain unclear. The study aimed to assess the differences in EH and functional status of the inner ear between patients with PD only and those with DEH.Method: Two PD and 10 DEH patients who had developed PD in early childhood were enrolled. Gadolinium-enhanced magnetic resonance imaging of the inner ear was performed in all patients to assess the degree of EH in the vestibule and cochlea.Results: There was no evidence of EH in the affected ears of patients with PD alone. DEH patients, however, showed significant EH in either the cochlea or vestibule, suggesting that EH development and DEH onset were strongly correlated.

Patterns of Long-term Hearing Loss in Hearing Preservation Cochlear Implant Surgery.

To describe patterns of hearing loss in patients with low-frequency residual hearing after cochlear implantation. Retrospective chart review. Tertiary referral center. Cochlear implant candidates with immediate postoperative residual low-frequency hearing. Hybrid or traditional cochlear implant. Audiograms to measure postoperative hearing. Of the 166 patients reviewed, 17 ears met the inclusion criteria. The age ranged from 3 years 2 months to 86 years. Etiology was unknown (n = 6), presbycusis (n = 5), genetic (n = 4), acoustic trauma (n = 1), and measles virus (n = 1). The Nucleus Hybrid S8 and S12 (n = 7) was the most common electrode array, and then Nucleus 422 (n = 6), Nucleus Contour Advance (n = 2), Med-El Flex 28 (n = 2), and Advanced Bionics Mid Scala (n = 1). Cochleostomy was performed in nine, and round window approach in nine patients. Average follow-up was 28 months (2-68 mo). Postoperative loss was mixed in eight and purely sensorineural in eight. The most common patterns of hearing loss were gradual decline (n = 7), and then fluctuating hearing (n = 6), stable (n = 3), and sudden loss (n = 1). One patient only had one postoperative audiogram. Some long-term hearing preservation was achieved in 94% of patients with immediate postoperative hearing preservation. Patients developed both mixed and sensorineural loss postoperatively. A majority of patients with mixed hearing loss had a supra-preoperative bone curve. Gradual decline and fluctuating hearing loss were the most common patterns of hearing loss; few patients had stable hearing and one had a sudden loss.

Meniere's disease.

Meniere's disease (MD) is a disorder of the inner ear that causes vertigo attacks, fluctuating hearing loss, tinnitus and aural fullness. The aetiology of MD is multifactorial. A characteristic sign of MD is endolymphatic hydrops (EH), a disorder in which excessive endolymph accumulates in the inner ear and causes damage to the ganglion cells. In most patients, the clinical symptoms of MD present after considerable accumulation of endolymph has occurred. However, some patients develop symptoms in the early stages of EH. The reason for the variability in the symptomatology is unknown and the relationship between EH and the clinical symptoms of MD requires further study. The diagnosis of MD is based on clinical symptoms but can be complemented with functional inner ear tests, including audiometry, vestibular-evoked myogenic potential testing, caloric testing, electrocochleography or head impulse tests. MRI has been optimized to directly visualize EH in the cochlea, vestibule and semicircular canals, and its use is shifting from the research setting to the clinic. The management of MD is mainly aimed at the relief of acute attacks of vertigo and the prevention of recurrent attacks. Therapeutic options are based on empirical evidence and include the management of risk factors and a conservative approach as the first line of treatment. When medical treatment is unable to suppress vertigo attacks, intratympanic gentamicin therapy or endolymphatic sac decompression surgery is usually considered. This Primer covers the pathophysiology, symptomatology, diagnosis, management, quality of life and prevention of MD.

A nationwide study on enlargement of the vestibular aqueduct in Japan.

To document the clinical features and associated pure-tone audiometry data in patients with enlargement of the vestibular aqueduct (EVA), and to identify risk factors for fluctuating hearing loss (HL) and vertigo/dizziness in EVA patients. In this nationwide survey in Japan, a first survey sheet was mailed to 662 board-certified otolaryngology departments to identify the ones treating EVA patients. A second survey sheet, which contained solicited clinical information and the results of the hearing tests, was mailed to all facilities that reported treating EVA cases. We analyzed clinical information, including age at the time of the most recent evaluation, gender, EVA side, age at onset, initial symptoms, precipitating factors, and etiology from survey responses, and assessed 4-frequency (500, 1000, 2000, and 4000Hz) pure-tone average (PTA) from accompanying pure-tone audiometry data. A multivariate logistic regression analysis was utilized to identify the possible risk factors for fluctuating HL and vertigo/dizziness. In total, 513 hospitals (response rate, 77.5%) responded to the first survey, and 113 reported treating patients with EVA. Seventy-nine out of the 113 hospitals (response rate 69.9%) responded to the second survey, and the data of 380 EVA patients were registered and analyzed. Of the 380 patients, 221 (58.2%) were female, suggesting female preponderance. The patient age ranged from 0 to 73 years (mean, 16.7 years; median, 13 years; interquartile range, 6-24 years). EVA was bilateral in 91.1% of the patients (346/380). The most prevalent initial symptom was HL (341/380), followed by vertigo/dizziness/imbalance (34/380). Sudden HL occurred secondary to head trauma in 5.3% of the patients and upper respiratory infection in 5.0%. Pure-tone audiometry showed profound HL (PTA >91dB) in 316 (52.0%) of the 608 ears in the 304 patients tested, and asymmetric HL, defined as >10dB, in 147 (48.4%) of the 304 patients. The mean PTA was 83.7dB (median, 91.3dB; interquartile range, 71.3-103.8dB), and the severity in PTA did not correlate with age. Multivariate logistic regression identified age ≥10 years (compared to age of 0-9 years), bilateral HL (compared to unilateral HL/normal hearing), a history of head trauma, and Pendred syndrome (compared to the other EVA-associated disorders) as significant risk factors for fluctuating HL and/or vertigo/dizziness. The present nationwide survey of 380 EVA patients provided a more precise description of the clinical features, including risk factors for fluctuating HL and vertigo/dizziness.

What is Menière's disease? A contemporary re-evaluation of endolymphatic hydrops.

Menière’s disease is a chronic condition with a prevalence of 200–500 per 100,000 and characterized by episodic attacks of vertigo, fluctuating hearing loss, tinnitus, aural pressure and a progressive loss of audiovestibular functions. Over 150 years ago, Prosper Menière was the first to recognize the inner ear as the site of lesion for this clinical syndrome. Over 75 years ago, endolymphatic hydrops was discovered as the pathologic correlate of Menière’s disease. However, this pathologic finding could be ascertained only in post-mortem histologic studies. Due to this diagnostic dilemma and the variable manifestation of the various audiovestibular symptoms, diagnostic classification systems based on clinical findings have been repeatedly modified and have not been uniformly used in scientific publications on Menière’s disease. Furthermore, the higher level measures of impact on quality of life such as vitality and social participation have been neglected hitherto. Recent developments of high-resolution MR imaging of the inner ear have now enabled us to visualize in vivo endolymphatic hydrops in patients with suspected Menière’s disease. In this review, we summarize the existing knowledge from temporal bone histologic studies and from the emerging evidence on imaging-based evaluation of patients with suspected Menière’s disease. These indicate that endolymphatic hydrops is responsible not only for the full-blown clinical triad of simultaneous attacks of auditory and vestibular dysfunction, but also for other clinical presentations such as “vestibular” and “cochlear Menière’s disease”. As a consequence, we propose a new terminology which is based on symptomatic and imaging characteristics of these clinical entities to clarify and simplify their diagnostic classification.

Auditory processing abilities in children with chronic otitis media with effusion

ABSTRACTConclusion The study results indicate that children with a history of otitis media with effusion (OME) suffer from auditory processing disorder to some degree. The findings support the hypothesis that fluctuating hearing loss may affect central auditory processing during critical periods.Objectives Evidence suggests that prolonged OME in children can result in an auditory processing disorder, presumably because hearing has been disrupted during an important developmental period. A lack of auditory stimulation leads to the abnormal development of the hearing pathways in the brain. The aim of the present study was to determine the effects of OME on binaural auditory function and auditory temporal processing.Method In the present study, the dichotic digit test (DDT) was used for binaural hearing, and the gap in noise (GIN) test was used to evaluate temporal hearing processing.Results The average values of GIN differed significantly between children with a history of OME and normal controls (p < 0.001). The mean values of the DDT score were significantly different between the two groups (p = 0.002).

Fluctuating Hearing Loss Responding to Corticosteroids: A Case of Cogan’s Syndrome Concomitant with Crohn’s Disease

Hearing loss has a broad differential diagnosis. Hearing impairment responding to corticosteroids is suggestive of an underlying autoimmune cause. We report a rare case of Cogan’s syndrome in a 28-year-old woman with no previous personal medical history. She had experienced two years of fluctuating hearing loss and bilateral keratitis that would respond to high-dose prednisone prescribed by her otorhinolaryngologist. We performed a complete work-up and ruled out most common causes of autoimmune inner ear diseases. Patient was diagnosed with Cogan’s syndrome based on clinical findings and prescribed corticosteroid-sparing immunosuppressive medication. During follow-up, she developed worsening fatigue, new onset anemia, and melena. Endoscopic and pathologic findings were diagnostic of Crohn’s disease. This case emphasizes the high prevalence of concomitant autoimmune diseases.

Intratympanic therapy in Meniere's syndrome or disease: up to date evidence for clinical practice.

Meniere's syndrome or disease (MS/D) is typically characterised by episodic vertigo, aural fullness, tinnitus and fluctuating hearing loss. There are multiple options available for treatment with variation in consensus on the best intervention. To evaluate the evidence on the efficacy of intratympanic therapies [steroids, gentamicin, antivirals and other therapies] on the frequency and severity of vertigo and other symptoms of MS/D. A literature search was performed on AMED, EMBASE, HMIC, MEDLINE, PsycINFO, BNI, CINAHL, HEALTH BUSINESS ELITE, CENTRAL and Cochrane Ear, Nose and Throat disorders group trials register using various MeSH. The search was restricted to English and human subjects, and the last date of search was December 2014. Randomised controlled trials of intratympanic therapies [steroids, gentamicin antivirals and latanoprost] versus a placebo or another treatment. We analysed 8 RCT's comparing intratympanic steroids, gentamicin, ganciclovir (antiviral) and latanoprost versus another form of intratympanic treatment or placebo. On the basis of 6 RCT's (n = 242), there is evidence to support the effectiveness of intratympanic steroids and gentamicin to control symptoms of vertigo in MS/D albeit with a risk of hearing loss with gentamicin. However, there was no consensus found on doses or treatment protocols. There was no evidence to support the use of other forms of intratympanic therapy (antivirals and latanoprost) in MS/D.

Progressive irreversible hearing loss is caused by stria vascularis degeneration in an Slc26a4-insufficient mouse model of large vestibular aqueduct syndrome

Hearing loss of patients with enlargement of the vestibular aqueduct (EVA) can fluctuate or progress, with overall downward progression. The most common detectable cause of EVA is mutations of SLC26A4. We previously described a transgenic Slc26a4-insufficient mouse model of EVA in which Slc26a4 expression is controlled by doxycycline administration. Mice that received doxycycline from conception until embryonic day 17.5 (DE17.5; doxycycline discontinued at embryonic day 17.5) had fluctuating hearing loss between 1 and 6months of age with an overall downward progression after 6months of age. In this study, we characterized the cochlear functional and structural changes underlying irreversible hearing loss in DE17.5 mice at 12months of age. The endocochlear potential was decreased and inversely correlated with auditory brainstem response thresholds. The stria vascularis was thickened and edematous in ears with less severe hearing loss, and thinned and atrophic in ears with more severe hearing loss. There were pathologic changes in marginal cell morphology and gene expression that were not observed at 3months. We conclude that strial dysfunction and degeneration are the primary causes of irreversible progressive hearing loss in our Slc26a4-insufficient mouse model of EVA. This model of primary strial atrophy may be used to explore the mechanisms of progressive hearing loss due to strial dysfunction.

Prognostic factors for sudden drops in hearing level after minor head injury in patients with an enlarged vestibular aqueduct: a meta-analysis.

To identify factors associated with sudden drops in hearing level after minor head trauma in patients with an enlarged vestibular aqueduct (EVA). A systematic review of the literature on sudden drops in hearing level after minor head trauma in patients with an EVA was conducted. The studies were retrieved from Embase, PubMed, CINAHL, and Cochrane and critically appraised using predefined criteria. Data on all described parameters were collected, and their relation with sudden drops after minor head trauma was statistically analyzed. Pooled data of 31 articles included 179 patients with 351 EVAs. Drops in hearing level after minor head trauma were experienced by 34% of the patients. We found a significant association between sudden deterioration of hearing after minor head trauma and preexisting fluctuating hearing loss (HL) (odds ratio, 8.6; p < 0.001; 95% confidence interval, 3.9-19.3). The diameter of the VA, type of preexisting HL, severity of HL, preexisting progressive HL, and the diagnosis Pendred syndrome were not significantly associated with sudden drops in hearing levels after head trauma. Only one-third of the patients with a proven EVA experienced sudden drops in hearing level because of head trauma. There is a significant association between preexisting fluctuating HL and the chance of sudden drops in hearing level caused by trauma. Stringent lifestyle advices, like avoiding activities with a risk of minor head trauma such as contact sports, might be restricted to patients with a fluctuating HL and those with a history of sudden drops on minor head trauma.

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The accurate diagnosis of inner ear anomalies on CT and/or MRI is of importance for understanding the pathogenesis of hearing impairment as well as balance disorders. Furthermore, it is possible to comprehend the molecular pathophysiology in some inner ear anomaly disorders in which a causative gene has been detected. Enlargement of the vestibular aqueduct (EVA) is the most common malformation of the inner ear. EVA can be observed in various disorders, including DFNB4/Pendred syndrome caused by SLC26A4 mutations, branchio-oto-renal/branchio-oto (BOR/BO) syndrome caused by mutations in EYA1, SIX1 or SIX5, and distal renal tubular acidosis caused by mutations in ATP6V1B1 or ATP6V0A4. SLC26A4 mutations can be responsible for EVA as well as incomplete partition type II and dilated vestibule. Characteristic phenotypes of EVA include fluctuating hearing loss and repetitive vertigo. However, such symptoms are not necessarily recognized in patients with the BOR/BO syndrome. The CHARGE syndrome is a disorder characterized by coloboma, heart defect, choanal atresia, retarded growth and development, genital hypoplasia and ear anomalies. Aplasia and hypoplasia of the semicircular canals are the most prevalent anomalies in the CHARGE syndrome. Other disorders associated with inner ear anomalies, including DFNX2 and the Usher syndrome, are mentioned in the review article.

Endolymphatic space size in patients with vestibular migraine and Ménière’s disease

Ménière's disease (MD) is characterized by episodic vertigo, fluctuating hearing loss and tinnitus. Vestibular migraine (VM) is a relatively new disorder that is characterized by episodic vertigo or dizziness, coexisting migraine and absence of hearing loss. It is occasionally difficult to distinguish between VM and vestibular MD with headache. Because endolymphatic hydrops (EH) is a characteristic sign of MD, we attempted to evaluate endolymphatic space size in both diseases. Endolymphatic space size in the vestibule and the cochlea was evaluated in seven patients with VM and in seven age- and sex-matched patients with vestibular MD. For visualization of the endolymphatic space, 3T magnetic resonance imaging was taken 4 h after intravenous injection of gadolinium contrast agents using three-dimensional fluid-attenuated inversion recovery and HYbriD of reversed image of positive endolymph signal and native image of positive perilymph signal techniques. In the vestibule of VM patients, EH was not observed, with the exception of two patients with unilateral or bilateral EH. In contrast, in the vestibule of patients with vestibular MD, all patients had significant EH, bilaterally or unilaterally. These results indicate that endolymphatic space size is significantly different between patients with VM and vestibular MD.

Slc26a4-insufficiency causes fluctuating hearing loss and stria vascularis dysfunction

SLC26A4 mutations can cause a distinctive hearing loss phenotype with sudden drops and fluctuation in patients. Existing Slc26a4 mutant mouse lines have a profound loss of hearing and vestibular function, with severe inner ear malformations that do not model this human phenotype. In this study, we generated Slc26a4-insufficient mice by manipulation of doxycycline administration to a transgenic mouse line in which all Slc26a4 expression was under the control of doxycycline. Doxycycline was administered from conception to embryonic day 17.5, and then it was discontinued. Auditory brainstem response thresholds showed significant fluctuation of hearing loss from 1 through 3months of age. The endocochlear potential, which is required for inner ear sensory cell function, correlated with auditory brainstem response thresholds. We observed degeneration of stria vascularis intermediate cells, the cells that generate the endocochlear potential, but no other abnormalities within the cochlea. We conclude that fluctuations of hearing result from fluctuations of the endocochlear potential and stria vascularis dysfunction in Slc26a4-insufficient mouse ears. This model can now be used to test potential interventions to reduce or prevent sudden hearing loss or fluctuation in human patients. Our strategy to generate a hypomorphic mouse model utilizing the tet-on system will be applicable to other diseases in which a hypomorphic allele is needed to model the human phenotype.

Differential diagnosis of peripheral vertigo

Dizziness can be classified mainly into 4 types: vertigo, disequilibrium, presyncope, and lightheadedness. Among these types, vertigo is a sensation of movement or motion due to various causes. The main causes of peripheral vertigo are benign paroxysmal positional vertigo (BPPV), acute vestibular neuritis (AVN), and Meniere’s disease. BPPV is one of the most common causes of peripheral vertigo. It is characterized by brief episodes of mild to intense vertigo, which are triggered by specific changes in the position of the head. BPPV is diagnosed from the characteristic symptoms and by observing the nystagmus such as in the Dix-Hallpike test. BPPV is treated with several canalith repositioning procedures. AVN is the second most common cause of peripheral vertigo. Its key symptom is the acute onset of sustained rotatory vertigo without hearing loss. It is treated with symptomatic therapy with antihistamines, anticholinergic agents, antidopaminergic agents, and gamma-aminobutyric acid-enhancing agents that are used for symptoms of acute vertigo. Meniere’s disease is characterized by episodic vertigo, fluctuating hearing loss, and tinnitus. It is traditionally relieved with life-style modification, a low-salt diet, and prescription of diuretics. However, diagnosis and treatment of the peripheral vertigo can be difficult without knowledge of BPPV, AVN, and Meniere’s disease. This article provides information on the differential diagnosis of peripheral vertigo in BPPV, AVN, and Meniere’s disease.

Congenital Cytomegalovirus Infection: Audiologic Outcome

The association between congenital cytomegalovirus (CMV) infection and sensorineural hearing loss (SNHL) was first described almost 50 years ago. Studies over the intervening decades have further described the relationship between congenital CMV infection and SNHL in children. However, congenital CMV infection remains a leading cause of SNHL in children in the United States and the world today. As more CMV infections are identified, it is important to recognize that infants who are born to seroimmune mothers are not completely protected from SNHL, although their hearing loss is often milder than that seen in CMV-infected infants following primary maternal infections. Late-onset and progressive hearing losses occur following congenital CMV infection, and CMV-infected infants should be evaluated regularly to provide for early detection of hearing loss and appropriate intervention. Fluctuating hearing loss that is not explained by concurrent middle ear infections is another characteristic of CMV-related hearing loss in children. Challenges still remain in predicting which children with congenital CMV infection will develop hearing loss and, among those who do develop loss, whether or not the loss will continue to deteriorate.

Can an Incomplete Ossicular Discontinuity Be Predicted by Audiometric and Clinical Findings?

To investigate a pathology of conductive hearing loss caused by an incomplete ossicular discontinuity. It can manifest as a triad of the following: 1) conductive hearing loss most prominent in the high frequencies (hfCHL), defined as [ABG for 4 kHz] > [mean ABG for 0.25-0.5 kHz] + 10 dB or more; 2) fluctuating hearing loss; and 3) short-lasting improvement of hearing after Valsalva maneuver. Retrospective clinical trial. Tertiary referral center. Fourteen patients with an incomplete ossicular discontinuity who underwent incus interposition were included. Incus interposition, mathematical model. First, the prevalence of the triad was documented. Second, the hypothesis that mechanical ossicular compliance was responsible for the triad of symptoms was evaluated and simulated in a mathematical model. Finally, the postoperative hearing results with a follow-up of 12 months were analyzed and compared with those reported in the literature. The presence of the triad of symptoms is a strong indicator for detecting patients with an incomplete ossicular discontinuity. High frequency conductive hearing loss was present in 93% (13/14 patients). Ten (71%) of the 14 patients presented with fluctuating hearing loss and improvement of hearing after Valsalva maneuver. The hfCHL could be simulated adequately in the mathematical model. Success rate for surgical intervention (ABG < 20 dB; 0.5, 1, 2, and 3 kHz) was 93% and was comparable to the results reported in the literature. Patients with hfCHL, fluctuating hearing loss, and improvement of hearing after Valsalva maneuver are likely to have an incomplete ossicular discontinuity. A favorable postoperative hearing recovery by incus interposition can be expected.