Total Deafness Research Articles

A Case of Pneumolabyrinth Induced by Eustachian Tube Air Inflation

Here, we report a case of pneumolabyrinth induced by Eustachian tube air inflation (ETAI) with a catheter and present evidence that multiple air bubbles entered the perilymphatic space through a preexisting oval window fistula. Tertiary referral center. Sixty-six-year-old woman. None. Air bubbles in the perilymphatic space revealed by cone beam computed tomography (CT) volume rendering imaging. The patient was referred to us because of vertigo, unsteadiness, and right hearing loss after ETAI using a Eustachian tube catheter. On Day 2, an audiogram showed right total deafness, and the perilymphatic space could not be identified on T2-weighted magnetic resonance imaging. A high-resolution cone beam CT scan obtained on Day 3 showed multiple air bubbles in the labyrinth. The volume rendering images clearly revealed a larger air bubble in the vestibule inside the footplate of the stapes and small air bubbles in the horizontal semicircular canal, superior semicircular canal, and basal and second turns of the cochlea. This finding indicates that the air bubbles entered the perilymphatic space through an oval widow fistula caused by a sudden elevation in intratympanic air pressure. Two months later, the air bubbles had disappeared, and the patient's high tone hearing had improved slightly. ETAI can cause a pneumolabyrinth if the intratympanic pressure rises beyond a certain critical level. In this situation, volume rendering imaging of high-resolution cone beam CT can be used to quantify and identify the air bubbles present. The images taken in this study suggest that air bubbles entered the perilymphatic space through a perilymphatic fistula.

Post-traumatic total deafness with normal CT scan

European Annals of Otorhinolaryngology, Head and Neck Diseases - Vol. 129 - N° 5 - p. 281-283

Vogt-Koyanagi-Harada syndrome (review of case reports)

AbstractVogt–Koyanagi–Harada (VKH) disease, also known as uveomeningitic syndrome, is a multisystem autoimmune disorder directed against any melanocyte-containing organ, including the eye, central nervous system, auditory, and integumentary system. We present a case of an African woman with total deafness and blindness of VKH, and discuss the histological characteristics, and review the classification and the management of VKH. The most important factor for prognosis is the immediate treatment. The rarity of this syndrome makes its diagnosis a challenge and the presence of deafness, tinnitus, and vertigo must be considered in its diagnosis.

Outcomes of Intratympanic Gentamicin Injection to Treat Ménière’s Disease

To investigate the outcomes of intratympanic (IT) gentamicin sulfate injection to treat intractable Ménière's disease (MD) using evidence-based methods. Data were retrieved from a MEDLINE search (January 1995 through January 2011). Selected were English-language articles presenting outcomes of IT gentamicin injection to treat MD that had a prospective study design and an evidence level of II or higher. The database was searched using Boolean combinations of the keywords intratympanic, gentamicin, and Ménière's disease. Dichotomous outcomes (success rate and total deafness) were expressed using a Mantel-Haenszel fixed-effects model. Pretreatment and posttreatment pure-tone audiometry results, and word discrimination scores were summarized using data synthesis techniques. A critical literature appraisal and meta-analysis shows that IT gentamicin injection can control vertigo in patients with MD who have limited hearing injury. The quality of research in this field has improved remarkably over the years. However, a large-scale randomized controlled trial is warranted to confirm the predictive factors for the effectiveness of IT gentamicin injection in treating MD.

Sporadic endolymphatic sac tumor: Its clinical, radiological, and histological features, management, and follow‐up

Sporadic endolymphatic sac tumor (ELST) is rare. We described the clinical, radiological, and histological features, treatment, and follow-up of ELST. This was a retrospective analysis of 7 cases of sporadic ELST that were managed between 1993 and 2010. Twenty-five to 75 years was the age range of the patients. Subjective hearing loss and tinnitus were the most common presenting features. Five patients had total deafness and 2 had severe sensorineural hearing loss. The most common radiological feature was temporal bone destruction with tumor extension to cerebellopontine angle and posterior cranial fossa. Cholesterol or hemosiderin cysts around the tumor could be a characteristic feature. Major skull base procedures were performed in all 7 cases, and complete tumor excision was achieved in 6 of them. One patient needed a second surgery after she was referred to us after an incomplete first surgery. Recurrences were detected in 2 patients during follow-up; 1 of them received irradiation without minimal change to the tumor size and the second refused any treatment for the recurrence. Both of them are alive with disease. Early detection and radical surgical excision at first attempt give best results. Radiotherapy could be considered only in unresectable recurrences.

Rosai Dorfman disease: Case with extensive dural involvement and cerebrospinal fluid pleocytosis

We report a young adult man who presented with chronic raised intracranial tension features and unusually progressive bilateral visual and hearing impairment of 18months duration. MR imaging showed extensive dural involvement and contiguous orbital and spinal disease. Cerebrospinal fluid demonstrated persistent high lymphocytic pleocytosis. Dural biopsy obtained from posterior cervical approach with C1 arch excision and meningeal biopsy revealed features of classical of Rosai–Dorfman disease. Histiocytes were strongly positive for CD-68 and S-100 proteins. The illness relentlessly progressed with patient developing total deafness and near total blindness at last follow-up.

Cochlear implantation for total deafness after ipsilateral ventriculoperitoneal shunt surgery: technical report

Cochlear implantation (CI) becomes more challenging when a programmable ventriculoperitoneal (VP) shunt is present on the same side of the head. We report a successful ipsilateral CI in a patient who suffered bilateral hearing loss after shunt surgery. We describe the CI case of a 49-year-old woman with an implanted VP shunt device, and perform a retrospective review of the clinical, audiological, and radiological features, surgical techniques, and postoperative functional outcomes. During CI, the magnet in the core of internal antenna of the CI device was positioned approximately 8 cm from the shunt valve so that it would not be affected by the magnetic field of the programmable valve. Although the CI antenna tip and shunt catheter overlapped, we were very careful not to pull the shunt catheter out of position while the musculoperiosteal flap was elevated. The present result suggests that ipsilateral CI is an option for deaf adult patients who have a programmable shunt.

Martwica popromienna kości skroniowej

Radiotherapy is an effective, modern method of treating malignant neoplasms of head and neck and also intracranial tumours. Technological development and computerization of equipment used in radiotherapy enabled introduction of modern methods of treatment allowing decrease the risk of postradiation complications but not eliminating them completely. Postradiant necrosis of temporal bone that is osteoradionecrosis (ORN) is a quite well known complication of radiotherapy of head and neck tumours. Secretory otitis media, conductive deafness, excretion of sequesters through external auditory canal, inflammation and atresia of external auditory canal are common findings during examination. Cases of disseminated osteoradionecrosis need to be qualified for operation due to potential intratemporal and intracranial complications. Radical excision of necrotic tissue gives possibility of parallel cochlear implantation among patients with total bilateral deafness, which is a consequence of radiotherapy. Aim of this study was clinical and epidemiological analysis of consequences and complications inside temporal bone which occurred after radiotherapy in the head and neck area. Conservative and surgical treatment possibilities according to progression of postradiant changes and severity of ailments were analyzed. Circumstances of conducting lateral petrosectomy with parallel cochlear implantation in case of osteoradionecrosis proceeding with total bilateral deafness were highlighted. Retrospective analysis of 12 patients treated in the Department of Otolaryngology at the Medical University of Warsaw in 2000–2010 for postradiant consequences and complications inside the temporal bone was performed. The diagnosis of osteoradionecrosis was based on clinical ENT examination including meticulous microscopic examination, audiologic evaluation and CT scans of temporal bone. The group consisted of 5 men and 7 women. In 8 patients changes were unilateral and in 4 – bilateral. In total, 16 cases (ears) of osteoradionecrosis in the area of temporal bone were analyzed. Patients were mainly complaining about hearing loss or deafness, tinnitus, otalgia, otorhoea, feeling of fullness and distension in the ear. Presence of effusion in the middle ear was an indication for ventilation tube insertion. Cases of ORN potentially endangered by further complications needed surgical treatment, therein lateral petrosectomy. Case of osteoradionecrosis proceeding with total bilateral deafness needed a concurrent cochlear implantation. Only the patients with diffuse osteoradionecrosis confirmed by CT scans ere qualified for surgery. Radiotherapy of head and neck tumours is charged with risk of complications, both early and appearing later after therapy. Osteoradionecrosis is found many years after radiation and the course of illness is repeatedly tricky and potentially life-threatening. In the face of complications resulting from the essence of illness surgical treatment, therein lateral petrosectomy is necessary. Complications of radiotherapy cannot eliminate it as a method of independent treatment nor combined with other methods.

Deafness after Skull Encephalic Trauma and Cochlear Implant

Objective: Total deafness may be among the consequences of a skull encephalic trauma with bilateral fracture of the temporal bone. The recovery of hearing function can occur with the application of a cochlear implant. The functional results will depend on the presence of neurological or neuropsychological alterations both morphological at cochlear level. Method: We present four cases of postverbal total deafness subsequent to single and/or bilateral fracture of the temporal bone who underwent cochlear implantation from 2000 to 2009. Two patients had monolateral anacusia with onset in childhood contralateral to the fracture, and 2 patients had injuries with bilateral fracture of the petrosal bone. Results: In all cases correct positioning of the electrode array was possible without complications after surgery, and at the activation all electrodes were working. Because of the greater encephalic damage and the reduced capacity of cortical integration, in 2 patients the spectral type of stimulation strategies was chosen with low rates of stimulation. One year after the activation of the perceptual-verbal ability all patients reached the recognition level in open set, and 3 patients were able to have telephone conversations with familiar voices. The patients with greater neurological complications also obtained significant benefits on the cognitive side. Conclusion: In these cases different variables affect the recovery of hearing function with the cochlear implant. CNS damage may be associated with fibrosis or ossification of the cochlea and limits to the proper insertion of the electrodes array. The neuropsychological and neurological complications, in our opinion, are not factors of exclusion.

An investigation into hearing loss among patients of 50 years or older

ObjectsTo investigate the extent of hearing loss in an elderly sample population to estimate hearing disorders among the age–equivalent population in China and to study primary clinical characteristics of presbycusis. MethodsClinical data from 110 hearing loss patients of both senium and pre–senium ages (95 males and 15 females, mean age = 74.4±12.1 years) were reviewed. Patients aged from 50 to 59 years were accepted as the pre–senium control group (n=15). The 95 senium patients (> 60 years of age) were divided into 4 groups according to age: 60+ group(60 to 69 years, n=25), 70+ group (70 to79 years, n=26), 80+ group (80 to 89 years, n=32) and 90+ group (90 years or older, n=12). Pure tone audiometry thresholds were measured in all 110 patients. Hearing loss severity of each tested ear was rated according to the Goodman classification criteria. Besides, audiometric configuration was examined in each ear. ResultsAudiometric testing showed normal hearing in 65 ears (29.5%), slight to moderately severe hearing loss in 131 ears (59.5%), and severe and profound loss in 24 ears (11%). Air–bone gaps were found in 12 ears (5.45%) indicating conductive hearing loss. Except the 12 ears with conductive hearing loss, audiograms showed gradually sloping loss in 99 ears (45%), sharply sloping loss in 34 ears (15.45%), flat loss in 45 ears (20.45%), notch pattern loss in 5 ears (2.27%), trough and rising pattern loss in 2 ears (0.91%), total deafness in 2 ears (0.91%), and normal hearing in 21 ears(9.55%). On average, hearing thresholds increased at a rate of approximately 10 dB per 10 year for subjects aged 60 and older. ConclusionsHearing thresholds tend to be stable in presbycusis patients aged from 50 to 70 years, increase significantly between 70 and 80 years of age, and reach another stable stage at high levels after 80 years of age, especially in high frequencies. Hearing loss in middle frequencies accounts for most of recession in loudness perception.

Patient satisfaction and functional results with the bone-anchored hearing aid (BAHA)

To assess patient satisfaction with bone-anchored hearing aids (BAHA) and the role of preoperative audiometric testing. A telephone satisfaction survey was conducted on all patients implanted between June 1, 2005 and February 1, 2008. Patients with unilateral total deafness underwent preoperative audiometric tests in quiet and in noise and stereoaudiometry with and without BAHA. Patients with a conductive hearing loss underwent preoperative audiometric tests in quiet and in noise and real-life testing at home using a headband. A standardized satisfaction questionnaire derived from the Entific BAHA questionnaire was used. Twenty-two out of 26 patients responded to the questionnaire. Ten patients were implanted for conductive hearing loss (CHL) and 12 for unilateral total deafness (UTD). Mean follow-up was 19 months in the UTD group and 21 months in the CHL group. Sixty-seven percent of UTD and 80% of CHL patients reported improved quality of life. The BAHA was worn for more than 4hours per day by 83% of UTD and 100% of CHL patients, and at least 5 days per week by 67% of UTD and 80% of CHL patients. BAHAs provided real benefit in all situations for CHL patients. In UTD, its benefit basically related to noisy environments. In UTD, satisfaction on preoperative stereoaudiometric testing in noise with and without BAHA was predictive of postimplantation satisfaction. In response to the question "Would you do it again?", 81% of patients answered "Yes".

E055 Cochlea implant care with Charge Syndrome

Partial deafness is characterised by normal or slightly elevated thresholds in the low-frequency band with nearly total deafness in higher frequencies. The aim of the study was to investigate whether the low-frequency hearing of partially deafened patients could be preserved after cochlear implant surgery applying Med-El Combi 40+ with partially inserted electrode. Cochlear implantation was performed in six partially deafened patients. Changes in auditory thresholds using pure tone audiometry recordings were measured to determine the extent of preservation of low-frequency hearing. Low-frequency hearing was preserved to the large extent in all patients. Preservation of low-frequency hearing creates the opportunity for application of electric and acoustic stimulation in the partially deafened. It could provide speech comprehension better than with either modality alone. Obtained results can be considered as the first step towards the application of cochlear implant in the treatment of partial deafness.

Labyrinthine window rupture as a cause of acute sensorineural hearing loss

Labyrinthine window rupture (LWR) is one cause of acute sensorineural hearing loss and need for early exploration is clear for good improved hearing. Acute sensorineural hearing loss of 60 dB or more treated from May 2006 to May 2010 were retrospectively analyzed. There were 21 ears of severe deafness, 18 ears of profound deafness, and 10 ears of total deafness. All patients were examined with temporal bone CT. Space-occupying lesions around the labyrinthine windows were suggestive images of LWR. Thirty-five ears were operated for LWR while 14 ears of SHL received conservative treatments. Fifty-seven percent of LWR improved 30 dB or more after sealing of both labyrinthine windows. Of the 15 markedly recovered ears, 14 ears were operated within 2 weeks from the onset. Of the five cured ears, four ears were operated within a week from the onset. As for the hearing prognosis of SHL, 88% of severe and profound deafness improved 30 dB or more but total deafness did not improve more than 30 dB. Exclusion of LWR from SHL and early surgical intervention in LWR will bring about good hearing prognosis to both LWR and SHL.

The patterns and risk factors of hearing loss following microvascular decompression for hemifacial spasm

The aim of this study was to reveal the risk factors including intraoperative brain stem auditory evoked potential (BAEP) changes and to define parameter and warning values of BAEP beyond which the probability of hearing impairment rises significantly. From April 1997 to February 2009, 1156 patients underwent microvascular decompression (MVD) for hemifacial spasm (HFS) and their medical records and audiologic data. The intraoperative BAEP monitoring was performed in all operations during surgery from the time of administration of general anesthesia until the time of skin closure. Pure tone audiometry (PTA) and Speech Discrimination Score (SDS) were performed on all patients before and after surgery for categorizing the patterns of hearing loss. There were 825 females and 331 males with a mean age of 48.7 years (range 17-75 years). The mean symptom duration was 67.8 months (range 1-420 months). At the 1-year follow-up examination, 1091 (94.4%) patients of the total 1156 patients exhibited a cured state, and 65 (5.6%) patients had residual spasms. Hearing loss occurred in 46 patients (3.9%). In 26 patients, PTA was decreased more than 15 dB with a proportional decrease of the SDS. In 10 patients, poor SDS without hearing loss occurred. Total deafness was developed in 10 patients. A higher incidence of BAEP change and a poor recovery especially amplitude in wave V during surgery was observed in patients with poor SDS (eight patients) and total deafness (seven patients) (p = 0.000). Reduction of amplitude more than 50% in wave V was a strong indicator for a worse outcome of the hearing capacity. The difference in other risk factors according to hearing loss pattern was not statistically significant (p > 0.05). Only female was significant (p = 0.005). The intraoperative BAEP change and a poorer recovery, especially reduction of amplitude more than 50% in wave V, was a strong indicator for a worse outcome of the hearing capacity. Vigilant intraoperative monitoring of the BAEP and adequate steps for recovery of the BAEP change could prevent hearing loss after MVD for HFS.

A Clinical Study of Hearing Loss Type in Idiopathic Sudden Sensorineural Hearing Loss

Our study of 677 cases of idiopathic sudden sensorineural hearing loss (ISSNHL) related to posttreatment recovery yielded the following results: 1) Most subjects—36.8%—had horizontal hearing loss 2) V character hearing loss curve was highest at 61.7% and lowest in high tone, near total deaf, and total deaf. 3) We classified 6 hearing loss types into 4 prognostic types—good for V character, intermediate for horizontal and reverse V, poor for high tone and near total deaf, extremely poor for total deaf. 4) Good recovery—a hearing level improvement of ≥30dB—was relatively high in near total deaf hearing loss. 5) Total deaf hearing loss had the highest vertigo at 68.4%.

The Effect of Ginkgo Biloba on the Survival of Spiral Ganglion Neurons in Rats

Background and ObjectivesZZGinkgo biloba extract (GBE) enhances cell survival in various organs. GBE protects nerve cells in the central nervous system and is clinically applied in Parkinson’s and Alzheimer’s disease. GBE can protect ototoxicity caused by cisplantin and gentamycin through rescue of hair cells in Organ of Corti and is accepted as one of the therapeutic agents for sudden deafness and tinnitus. The experimental study on GBE for the inner ear is confined to the hair cells, not to the spiral ganglion neurons (SGNs) which is the stimulated part by the electrode of cochlear implant. The aim of this study is to elucidate the effect of GBE on the survival of SGNs after hair cell loss in rats. Materials and MethodZZTen Sprague-Dawley rats aged 50 days (P50) were deafened with kanamycin sulfate. GBE (EGb 761) was injected into the right cochlea and artificial peri lymph was injected into the left side. The number and size of SGNs were compared after immunohistochemical statin in both groups. The expression of pJun, which is well-known as a proapoptotic transcription factor in the cochlea, was also compared. ResultsZZThe number of SGNs was significantly larger in the GBE group than the control. The expression of pJun activity was significantly decreased in GBE group than the control. The size of SGNs in both groups was similar. ConclusionZZThese results suggest that GBE can protect SGNs death by inhibiting the pJun-C-jun N-terminal kinase pathway. GBE might be a potential drug for the patients with total deafness before or after cochlear implantation surgery for better hearing results. � Korean J Otorhinolaryngol-Head Neck Surg 2011;54:526-31

Vestibular Schwannoma and Fitness to Fly

When a pilot is referred for vestibular schwannoma (VS), his or her fitness to fly may be questioned. The objective of this retrospective study was to describe a series of VS cases in a pilot population and to discuss their fitness to fly options. Between September 2002 and March 2010, the ENT/Head and Neck Surgery Department of the National Pilot Expertise Center conducted nearly 120,000 expert consultations for 40,000 pilots. We examined the files of 10 pilots who were referred to our 2 national experts for VS. At the time of the expert consultation, hypoacusis was present in nine cases (four with total deafness), tinnitus in one case, and vertigo in nine cases. In our series, only 2 of the 10 pilots experienced a negative impact on their fitness to fly. Decisions on fitness to fly were based on several factors: minimally disturbed audition, i.e., less than a 35-dB hearing loss with a good speech discrimination score; good balance, i.e., no reported difficulties; no spontaneous nystagmus recorded on videonystagmography (VNG); no postural deviation; and a normal head-shaking test. The delay and the VS's evolution between diagnosis and expert consultation are important because the selection of a treatment to control VS is critical in minimizing the possible associated complications. When a pilot is referred for VS, his or her fitness to fly is determined by the size of the tumor, balance, auditory status, and the follow-up results of these findings. The complications that may arise from VS treatments must also be considered.

Autoimmune Inner Ear Disease- A Clinical Viewpoint

Recent developments in medicine have given us a better insight into a group of disorders known as autoimmune diseases. In particular, advances have occurred in our understanding of the Autoimmune Inner Ear Disease (AIED). In this article, the authors review the different postulated theories in the pathogenesis of this disease. The clinical presentation, the available para-clinical diagnostic tools, and the important differential diagnoses will be summarized. The management methods, including steroid therapy, immunosuppressive medications, other biological agents and intra-tympanic injections, will be addressed. Cochlear implantation as a final solution to the advanced stages of the disease, causing total deafness, will also be discussed.

Prevalence of Acoustic Neuroma Associated with Each Configuration of Pure Tone Audiogram in Patients with Asymmetric Sensorineural Hearing Loss

The criteria have not yet been established for identifying the configuration of a pure tone audiogram constituting abnormal results that warrant further investigation. The purpose of this study was to determine the prevalence of acoustic neuroma associated with each configuration of the pure tone audiogram in patients with asymmetric sensorineural hearing loss (SNHL). We performed a retrospective chart review of 500 patients 15 years of age or older who had asymmetric SNHL and had undergone magnetic resonance imaging. The prevalence of acoustic neuroma in these patients was 2.6% (13 of 500). The prevalence of acoustic neuroma in each audiometric configuration was as follows: 7.1% (3 of 42) for a basin-shaped loss (odds ratio [OR] versus overall prevalence, 2.88; p = 0.23; 95% confidence interval [CI], 0.79 to 10.54), 4.7% (5 of 107) for a flat loss, 3.4% (2 of 58) for total deafness, 2.9% (1 of 34) for a high-frequency sloping audiogram, and 2.5% (2 of 81) for a high-frequency steep audiogram. The prevalence in patients with nonimproving idiopathic sudden deafness was 8.1% (OR, 3.29; p = 0.06; 95% CI, 1.13 to 9.55). In conclusion, 2.9% to 8.1% of patients with a characteristic configuration of the pure tone audiogram and symptoms of nonimproving or progressive idiopathic sudden deafness may have acoustic neuroma.

A case of cochlear implantation in a patient with Paget disease

Paget disease is a chronic osteoclastic disorder of unknown etiology that occurs most frequently in the spine, pelvis, and skull. Excessive bone resorption and consequent secondary bone formation are the main pathology associated with this condition. Paget disease is common in Europe and North America, but relatively uncommon in Asia. There is only one case report of Paget disease of temporal bone in Korea [1]. Seventy percent of Paget patients have skull lesions, and half of them have hearing impairment [2]. The type of hearing loss includes conductive, sensorineural, or mixed varieties; however, total deafness is not common. There is only one prior case report of cochlear implantation in a patient with Paget disease [3]. Here, another case of cochlear implantation in a patient with Paget disease is reported.