Mixed-type Hearing Loss Research Articles

Assessment of auditory functions in patients with hepatic glycogen storage diseases.

Hepatic glycogen storage diseases are a group of diseases manifesting mainly with hypoglycemia and hepatomegaly. The patients require frequent daytime and nocturnal feedings. Hypoglycemia may cause sensorineural hearing loss and nocturnal feeding is a risk factor for the development of gastroesophageal reflux that may cause chronic otitis media and hearing loss consequently. We aimed to determine the prevalence and characteristics of hearing loss in hepatic glycogen storage diseases. A total of 24 patients with hepatic glycogen storage disease (15 glycogen storage disease type I and 9 non type I) and 24 age/sex matched healthy controls were enrolled in the study. Pure tone audiometer, immitansmetry, acoustic reflex measurement, otoacoustic emission test (OAE) and auditory brainstem response (ABR) tests were applied to all participants. Hearing loss was determined in 17/24 patients (12 glycogen storage disease type I and 5 non type I) with pure tone audiometer. Interpretation of all the findings revealed a total of 8 patients had conductive and 9 had mixed hearing loss. All parameters were significantly different than the control group. This is the first study to comprehensively assess the auditory functions of patients with hepatic glycogen storage disease. Audiological findings determined a significantly increased prevalence of conductive/ mixed type hearing loss in the patient group which is a new finding in the literature. Further studies with extended patient numbers are required to enlighten the underlying pathophysiology.

Superior Semicircular Canal Dehiscence Syndrome Manifested as Menière’s Disease: A Case Report

The absence of a temporal bone overlying the superior semicircular canal causes superior semicircular canal dehiscence (SSCD). The vestibular symptom of SSCD syndrome (SCDS) is vertigo and audiologic symptoms include autophony, hyperacusis, and ear fullness. A 52-year-old man presented with left-sided unilateral hearing loss, aural fullness, and recurrent spinning-type vertigo. He had positive Hennebert sign and mixed-type hearing loss, with a prominent low-frequency air-bone gap. These symptoms reminded us of SCDS, and computed tomography (CT) revealed SSCD. However, the patient had not experienced vertigo until 1 week prior to the visit. In addition, the audiogram revealed fluctuation of hearing, which was aggravated when the vestibular symptoms manifested. Vertigo might be due to Menière’s disease rather than SCDS and SSCD was incidentally detected on CT. According to reviews, this is no reported case of SCDS manifested as Menière’s disease, so we report this case with a brief review of the literature.

Hearing loss in patients with head and neck cancer post chemoradiotherapy

<p class="abstract"><strong>Background:</strong> The aim of the study was to study incidence of sensorineural hearing loss (SNHL) and conductive hearing loss (CHL) in patients receiving chemoradiation for head and neck malignancies and to ascertain the pattern of hearing loss in different frequencies.</p><p class="abstract"><strong>Methods:</strong> The present study was conducted on 100 patients of histopathologically proven head and neck malignancy admitted in Department of Otorhinolaryngology and head and neck surgery, SMGS Hospital, GMC Jammu during a time period of July 2017 to June 2019.Inclusion criteria for our study were patients with primary site of malignancy at nasal cavity, nasopharynx, oral cavity, oropharynx, larynx, hypopharynx and salivary glands undergoing chemo-radiotherapy. Hearing loss was calculated with reference to the pre-treatment hearing threshold. A decrease of >20 dB in single frequency or >10 dB in two or more consecutive frequency was considered significant. </p><p class="abstract"><strong>Results:</strong> In the study, 65 patients (65%) developed sensorineural hearing loss and 22 patient (22%) developed conductive hearing loss during course of treatment. However, 7 patients (7%) developed a mixed type hearing loss during course of treatment.</p><p class="abstract"><strong>Conclusions:</strong> Although chemo radiation affects all the frequencies significantly but SNHL is markedly seen in higher frequencies. Conductive hearing loss is also a common adverse event after radiotherapy to the upper head and neck area.</p>

Osteogenesis imperfecta and hearing loss: an analysis of patients attended at a benchmark treatment center in southern Brazil

To characterize the audiological findings of a sample of patients with osteogenesis imperfecta (OI) in southern Brazil. This was a cross-sectional, observational, quantitative study. Research was carried out at a hospital which is considered to offer benchmark treatment for patients with OI in southern Brazil. Seventy-seven patients were recruited, at ages between 5 and 55years; the mean age was 21.9 ± 14.3years. Patients were divided into three age groups: 10 and under, 10-19 and over 19. During our study, peripheral audiological assessments were performed (pure tone testing and acoustic immittance measurements). The main outcome measures taken into account were airway thresholds, bone conduction, air-bone gap and compliance values between compared frequencies. Data were analyzed per ear. Normal hearing thresholds were found in 96 (64.4%) ears of the total sample. When analysis was stratified into age groups, normal hearing thresholds were found in 81.3%, 65%, and 54.4%, of the children, adolescent and adult groups, respectively. Concerning hearing impairments, there was a predominance of mixed type hearing loss in adults (21.1%) whereas adolescents presented conductive hearing loss or a conductive loss factor, while maintaining airway thresholds within the bounds of normality (30%). Ears with hearing loss showed superior compliance meansthan ears without hearing loss (p = 0.002). Overall, the majority of the subjects in this patient sample presented normal hearing thresholds. When present, hearing impairments were more prevalent in the adult group than in the adolescent or children's groups.

Does Tympanic Membrane Perforation Have a Protective Affect on the Inner Ear in Blast-Injured Patients?

Background Blast-induced hearing loss is an acoustic trauma commonly caused by high-energy explosions of improvised explosive devices, and the auditory system may be affected by blast damage. This study aims to evaluate the protective effect of tympanic membrane perforation (TMP) on the inner ear against blast injury. Methods In this study, 43 adult patients who had suffered blast injury were divided into three subgroups: intact tympanic membranes in both ears, unilateral TMP, and bilateral TMP. Each patient underwent a comprehensive audiogram, including bone conduction, in the audiology department. Results Evaluation was performed on 43 (100%) males with a mean age of 31.44±8.01 years (range, 18-52 years). When the type of hearing loss was evaluated separately for each ear, sensorineural hearing loss (SNHL) was observed in 31 (36%), high-frequency SNHL in 26 (30.2%), conductive hearing loss in eight (9.3%), and mixed type hearing loss in 21 (24.4%) ears. TMP was detected in 21 (48.8%) of 43 blast-injured patients, on the right side in four (9.3%) patients, on the left side in seven (16.3%), and bilateral in 10 (23.3%). When the type of acoustic trauma was evaluated, 15 (34.9%) patients were observed to have suffered from the explosion of an IED, 12 (30.2%) from weapon explosion, six (14%) were a vehicle bomb explosion, three (7%) were projectile missile explosion, three (7%) were mortar explosion, two (4.7%) were mine explosion, and two (4.7%) were exposed to the explosion in an armored vehicle (Table 1). Conclusion No significant difference was observed in the majority of the frequencies whether the tympanic membrane was perforated or not in the blast-injured patients and it was concluded that tympanic membrane perforation caused by blast injury had no protective effect on the inner ear.

Cochlear Nerve Hypoplasia: Audiological Characteristics in Children and Adults

Background: Cochlear nerve deficiency is a general term used to describe both cochlear nerve hypoplasia (CNH) and cochlear nerve aplasia. Although these two conditions can have similar results on audiological evaluation, CNH yields more variation in audiological tests. Objectives: To describe the audiological characteristics of the CNH cases in our series in relation to radiological findings. Methods: We reviewed the medical charts, audiological findings, and radiological findings on cases with CNH. We included cases with CNH in one ear or both ears. Out of 90 subjects with CNH, we included a total of 40 individuals (21 women and 19 men; 49 ears) in the current study. We reviewed and analyzed the participants’ audiological test results according to the radiological findings. Results: Cases with CNH showed variations according to the cochlear structure. There were 13 normal cochleae, 4 with incomplete partition type I, and 32 with cochlear hypoplasia. The accompanying cochlear apertures also showed variation: 17 were normal, 28 stenotic, and 4 aplastic cochlear apertures. The subjects displayed hearing loss ranging from moderate to profound; furthermore, 4 subjects had no response to sound whatsoever. The degree of hearing loss was not statistically significantly different with regard to the presence or absence of cochlear malformation with CNH (p > 0.005). We observed both sensorineural hearing loss and mixed-type hearing loss among the CNH cases. Conclusions: CNH is the presence of a cochlear nerve that is smaller in diameter than the facial nerve. It can be accompanied with other associated inner ear malformations of different degrees of severity. We observed degrees of hearing loss ranging from moderate to profound.

Evaluation of Hearing and Balance Functions of Patients with Sickle Cell Anemia

Introduction: Sickle cell anemia is a disease characterized by a wide vaso-occlusive incident from micro-vascular incident to muscularactivity. The cochlear function can also get affected by this vaso-occlusion. Objective: It is aimed at determining what kind of effects sickle cell anemia has on hearing and balance system. Methods: This study has been conducted on 46 patients with sickle cell anemia and 45 healthy individuals. For all participants, their pure tone hearing thresholds and videonystagmography (VNG) findings have been determined in 17 frequencies between 125–16.000 Hz. Results: All hearing thresholds between 125 and 16,000 Hz, pure tone averages of patients with sickle cell anemia have been found statistically significant to be higher than the corresponding values in the control group(p < 0.05). The normal hearing rate of patients with sickle cell anemia has been determined to be 71.1% conductive hearing loss (CHL) to be 4.4%, sensorineural hearing loss (SNHL) to be 22.2%, and mixed type hearing loss to be 2.2% in right ear; the normal hearing rate has been determined to be 71.1%, CHL to be 2.2%, SNHL to be 22.2%, and mixed type hearing loss to be 4.4% in left ear. Statistically significant difference has not been found between head shake, spontaneous nystagmus, optokinetic, tracking test batteries, static and dynamic positional tests used in VNG, saccade accuracy and saccade peak velocity, which are saccadic test findings of 2 groups. However, saccadic latency, which is a saccadic test finding, has been determined to be longer in patients with sickle cell anemia in comparison to the control group. Conclusion: While sickle cell anemia causes hearing deficits, it does not have any effect on the central or peripheral vestibular system.

Temporal Bone Posterior Wall Defect, a New Clinic Entity?

To define clinical and audiological findings in patients with temporal bone posterior wall defects (TBPWD) and to investigate possible relationships between these findings and the characteristics of the defect. The computed tomography (CT) views of 1198 patients with vestibulocochlear symptoms between 2007 and 2012 were retrospectively evaluated, and TBPWD and associated anomalies were investigated. Patients who had TBPWD were called back, and clinical and audiological examinations (tympanometry, pure tone audiometry, acoustic reflexes, and otoacoustic emission) were performed. Twenty-eight (2.34%) patients had TBPWD. Twenty-three of them were eligible for the study. Size of the defect was significantly correlated with the presence of tinnitus and/or vertigo (p<0.005). The cut-off values for the largest size of TBPWD were 1.65 mm [sensitivity: 0.67 and specificity: 0.77 (95% CI: 0.58-0.97); p=0.04] in case of the presence of tinnitus and 1.85 for vertigo (sensitivity: 0.78 and specificity: 0.86 (95% CI: 0.67-0.99); p=0.006). In pure tone audiometry tests, mixed-type hearing loss was present in four (17%) patients, sensorineural hearing loss was present in three (13%) patients, and conductive-type hearing loss was present only in one (4%) patient. Otoacoustic emission tests revealed significant differences in signal/noise ratios at frequencies of 500, 750, 1000, and 6000 Hz. For the first time in the literature, we defined clinical and audiological findings in patients with TBPWDs. These defects seem to cause more prevalent symptoms of vertigo and tinnitus and disturb the audiological characteristics of patients.

Isolated Sensorineural Hearing Loss as a Sequela after Lightning Strike.

In most of the surviving patients after a lightning strike, audiovestibular abnormalities have been reported. The most frequently reported type of abnormalities is a tympanic membrane perforation with hearing loss and external ear canal burn. However a sensor neural hearing loss and mixed type hearing loss can also occur, but these occur rarely. A nineteen-year-old female patient had, after a lightning strike, serious burns on the left ear, behind the ear, and on the chest and neck. She also had in her left ear 108 dB hearing loss with irregular central perforation and in her right ear 52 dB sensorineural hearing loss. There was no hearing loss before the strike. A hearing aid was recommended for the right ear and good care and follow-up were recommended for the left ear. A lightning strike can cause serious audiological damage. Therefore, it is necessary to make a careful audiovestibular evaluation of the patients. Although there exist rarely healed cases from sensorineural hearing loss after lightning strike in literature, in our case hearing loss occurred bilaterally and then it healed unilaterally. This condition is quite rare in literature.

Malignant Melanoma of the Temporal Bone Initially Presenting with Dizziness

A 48-year-old male presented to the otolaryngology outpatient clinic with a 3-month history of dizziness. Physical examination showed horizontal nystagmus toward the left on left lateral gaze and an intact tympanic membrane. No other neurological abnormalities were observed in the neurology department consultation. Thus, vestibular neuritis was suspected, and conservative treatment (dimenhydrinate, flunarizine, and ginkgo biloba leaf extract) was thus carried out. On follow-up after 1months, the dizziness symptom was maintained. Caloric tests were underwent and demonstrated right-sided unilateral weakness of 70%. On the ENT outpatient department after 2 months, the symptoms had relapsed and right pulsatile tinnitus and diplopia were noted. Puretone audiometry showed mild, mixed-type hearing loss in the right ear. Computed tomography (CT) of the temporal bone showed a soft tissue density from the right mastoid to the petrous apex with bony erosion. The lesion extended to the middle ear cavity at the cochlear promontory (Fig. 1). Magnetic resonance imaging (MRI) revealed an approximately 4.5 cm, irregularly contoured mass centered at the right petrous apex. The mass showed iso-to-low signal intensity on T2-weighted images and heterogeneous high-signal Malignant Melanoma of the Temporal Bone Initially Presenting with Dizziness

Stapes Surgery Outcomes: The Practice of 35 Years

Stapes Surgery Outcomes: The Practice of 35 Years

Pattern of hearing loss following cochlear implantation.

Cochlear implantation is associated with deterioration in hearing. Despite the fact that the damage is presumed to be of sensory origin, residual hearing is usually assessed by air-conduction thresholds alone. This study sought to determine if surgery may cause changes in air- and bone-conduction thresholds producing a mixed-type hearing loss. The sample included 18 patients (mean age 37 years) with an air-bone gap of 10 dB over three consecutive frequencies and measurable masked and reliable bone-conduction thresholds of operated and non-operated ears who underwent cochlear implant surgery. All underwent comprehensive audiologic and otologic assessment and imaging before and after surgery. The air-bone gap in the treated ears was 17-41 dB preoperatively and 13-59 dB postoperatively over 250-4,000 Hz. Air-conduction thresholds in the treated ears significantly deteriorated after surgery, by a mean of 10-21 dB. Bone-conduction levels deteriorated nonsignificantly by 0.8-7.5 dB. The findings indicate that the increase in air-conduction threshold after cochlear implantation accounts for most of the postoperative increase in the air-bone gap. Changes in the mechanics of the inner ear may play an important role. Further studies in larger samples including objective measures of inner ear mechanics may add information on the source of the air-bone gap.

Risk of Developing Sudden Sensorineural Hearing Loss in Patients with Acute Otitis Media: A Multicenter Retrospective Analysis

Objective: The aim of the study was to determine the etiology of Sudden Sensorineural Hearing Loss (SSHL) and to call attention to Acute Otitis Media (AOM) with SSHL. Study Design and Setting: We conducted a retrospective, multicenter analysis of SSHL. We were used spearman correlation matrix test for correlations between all variables. One hundred twelve patients with SSHL were evaluated. Results: A total of 112 patients (62 males, 50 females) ranging in age from 17 to 70 years (average male 40.21 ± 14.04, average female 40.26 ± 11.16) were included. Fourteen of these had AOM. The majority of patients had moderate hearing loss. Flat and down-sloping types of audiogram were also observed (P 0.05). Otoscopic examination was consistent with AOM. SSHL occurred as mixed-type hearing loss. Tympanometry was observed as type A. Conclusion: In the treatment and follow-up periods, AOM patients should be checked and treated for the presence (if any) of early hearing loss.

Functional and structural evaluation of hearing in acromegaly

The impact of acromegaly on the auditory system remains unknown. This study aimed to examine audiological symptoms and the structure and function of the auditory system in patients with acromegaly. DESIGN/SETTING AND PATIENTS: The study included 44 patients with acromegaly. Pure tone audiometry, speech audiometry, tympanometry, otoacoustic emissions testing, computerized tomography of the temporal bone and magnetic resonance imaging of the ear were performed in all patients. The study also included 36 age- and sex-matched healthy controls. The patients with acromegaly were divided into three subgroups, as follows: controlled disease (n=13); partially controlled disease (n=16); uncontrolled disease (n = 15). In all, 43% of the patients with acromegaly had hearing loss, and 20% had had an episode of otitis in at least one ear. Median pure tone average (PTA) in the patients with acromegaly was 12.5 dB (range: 2-72 dB), vs 8.3 dB (range: 0-20 dB) in the control group (P < 0.001). PTA did not differ significantly between the three patient subgroups. Audiometric tests showed various degrees of hearing loss in at least one ear in 21 patients (48%). Conductive, sensorineural and mixed type hearing loss in at least one ear was noted in 9%, 30% and 18% of the patients, respectively. Auditory imaging showed that 50% of the patients had temporomandibular joint degeneration (TMJD). Hearing loss is quite common in patients with acromegaly. Contrary to common belief, in this study, conductive hearing loss did not occur more frequently than other types. Based on PTA findings in the controls and patients with acromegaly, acromegaly caused hearing loss, but the level of disease activity had no effect on hearing. Middle ear pressure problems might be caused by increased perilymph because of growth hormone (GH)-related volume overload.

Cochlear otosclerosis

The aim of this study is to summarize current advances in research and clinical aspects of cochlear otosclerosis. Recent studies have revealed that otosclerosis is a process of bone remodeling that is unique to the otic capsule only. Even though no obvious bone remodeling is seen in the otic capsule under normal conditions, remodeling starts when some molecular factors trigger the capsule in certain patients who have genetic and/or environmental tendencies. Cochlear otosclerosis is defined as otosclerosis located in the otic capsule involving the cochlear endosteum and causing sensorineural hearing loss or mixed-type hearing loss. It has been clearly shown that, when otosclerosis is sufficiently severe to involve the cochlear endosteum, it usually fixes the stapes as well.

Fluctuating mixed-type hearing loss associated with synovitis–acne–pustulosis–hyperostosis–osteomyelitis (SAPHO) syndrome

Synovitis, acne, palmoplantar pustulosis (PPP), hyperostosis, and osteitis (SAPHO) are the characteristic features of SAPHO syndrome. A 53-year-old woman had been treated for PPP for 2 years. She complained of hearing loss in the right ear, and otitis externa was diagnosed. The pure-tone audiogram (PTA) indicated mild hearing loss in the right ear, and her hearing continued to deteriorate despite recovery from inflammation. Her tympanogram was of the As-type, and acoustic reflex was absent in the right ear. A computed tomography (CT) scan revealed bilateral normal ossicles and cochleas. Bone scintigraphy revealed tracer uptake in the bilateral sternoclavicular joints, glenohumeral joints, and the capital humerus. She was hospitalized for arthralgia, and the pain was controlled with steroid therapy. Her right hearing deteriorated soon after the tapering of the steroid; her hearing recovered after cyclosporine therapy was initiated. The first tonsil provocation test showed increased blood cells in the urine, and the second test showed exacerbation of pustulosis. Despite immunosuppressant therapy, the arthritis attacks and hearing loss persisted; therefore, tonsillectomy was performed, which improved PPP. However, her hearing remained unchanged after the operation. We considered that irreversible changes might have already developed in the ossicular joints, and ossicular reconstruction was performed. Thereafter, her hearing and earache improved.

A patient with ascending aortic dilatation, similar to phenotypes of connective tissue disorders

We report on the clinical and molecular findings of a patient who presented alopecia, epicanthus, micrognathia, retrognathia, high arched palate, hypertelorism, Chiari type I malformation, mixed-type hearing loss but with normal heartbeat Q-T interval, malformed earlobes, down-slanted palpebral fissures, downturned corners of the mouth, syndactyly, atopic eczema, and seizures. The patient was a male adult, 23 years old, with short stature (153 cm) and low weight (50.5 kg), due to severe aortic insufficiency and dilatation of the ascending aorta. Conventional cytogenetic screening did not show any chromosomal gains or losses. Molecular genetic screening was conducted for gene mutations involved in various syndromes; the mutations found included [beta-fibrinogen -455 G>A wt/wt (wt/mut), PAI-1 4G/5G (4G/4G), HPA1 a/b (a/a), MTHFR C677T wt/wt (wt/mut), ACE I/D (I/I), and Apo E E3/E4]. Many clinical and molecular genetics findings overlapped with other conditions associated with arterial tortuosity and arterial aneurysms, including the Marfan, Ehler-Danlos, Shprintzen-Goldberg, and Loeys-Dietz syndromes. Although a diagnosis of Shprintzen-Goldberg syndrome was based on clinical findings and radiographic findings indicate other syndromes, aortic root dilatation seems to be a new symptom, similar to phenotypes of connective tissue disorders. The unique grouping of clinical manifestations in this patient and the molecular genetics findings lead us to suggest that this case could be an example of a previously unrecognized syndrome.

Progressive Mixed Type Hearing Loss in an Adult Male with Osteopetrosis

• Notably: • There are two case series in the Otolaryngology literature, but both are restricted to the infant and juvenile forms of the disease. • There is one large case series relating to the adulttype disease, but it is in the Orthopedics literature and only mentions hearing loss in passing. • There are two similar case reports in the international Otolaryngology literature relating to adulttype osteopetrosis and its otologic consequences.

Effect of an enlarged endolymphatic duct on bone conduction threshold

Conclusion. An enlarged endolymphatic duct and sac (EDS) that makes contact with the cerebrospinal fluid–dural interface plays an important role in the pathway of bone conduction and enhances bone conduction at lower frequencies. Objectives. We investigated whether the bone conduction threshold was improved when the EDS was enlarged. Subjects and methods. Twenty-three patients (46 ears) with large vestibular aqueducts underwent standard pure tone audiometry (PTA) and magnetic resonance imaging (MRI) to investigate the relation between the diameter of the endolymphatic duct (ED) and the air or bone conduction threshold. We also investigated the relation between the volume of the EDS and the air or bone conduction threshold. Results. All ears had a mixed type hearing loss. The air–bone gaps were significantly larger at 250 and 500 Hz than at higher frequencies. The bone conduction thresholds were significantly lower at 250 Hz and 1000 Hz when the diameter of the ED was large, whereas there was no relation between the diameter of the ED and the air conduction threshold. In addition, there was no correlation between the volume of the EDS and air or bone conduction thresholds.

Angiomatous type of jugular foramen meningioma with neck extension: Differential diagnosis from paraganglioma and schwannoma

Meningiomas involving the jugular foramen and parapharyngeal space are extremely rare. They most commonly occur intracranially and then extend to the extracranial region through the foramen of the skull base, such as jugular foramen. Clinically, these tumors mimic the more common glomus jugulare tumor. Preoperative diagnosis can be correct on the basis of the characteristic imaging findings. A 52-year-old woman was seen with a left neck mass and mixed-type hearing loss. She underwent physical examination, MRI, high-resolution CT, and angiography. Physical examination revealed a retro-tympanic, pulsatile red mass in the left ear, and mild bulging of the left oropharyngeal wall. The patient was found to have the spreading, carpet-like, meningioma with extracranial extensions via jugular foramen to parapharyngeal space. Preoperative imaging strategy allowing accurate preoperative diagnosis is discussed. Accurate distinction between meningioma and glomus tumor or schwannoma is possible in most cases, with attention to fine radiologic detail.