Tonic tensor tympani syndrome in tinnitus and hyperacusis patients: a multi-clinic prevalence study.

UNLABELLED: Tonic tensor tympani syndrome (TTTS) is an involuntary, anxiety-based condition where the reflex threshold for tensor tympani muscle activity is reduced, causing a frequent spasm. This can trigger aural symptoms from tympanic membrane tension, middle ear ventilation alterations and trigeminal nerve irritability. TTTS is considered to cause the distinctive symptoms of acoustic shock (AS), which can develop after exposure to an unexpected loud sound perceived as highly threatening. Hyperacusis is a dominant AS symptom. Aural pain/blockage without underlying pathology has been noted in tinnitus and hyperacusis patients, without wide acknowledgment. This multiclinic study investigated the prevalence of TTTS symptoms and AS in tinnitus and hyperacusis patients. This study included consecutive patients with tinnitus and/or hyperacusis seen in multiple clinics. DATA COLLECTED: Symptoms consistent with TTTS (pain/numbness/burning in and around the ear; aural "blockage"; mild vertigo/nausea; "muffled" hearing; tympanic flutter; headache); onset or exacerbation from exposure to loud/intolerable sounds; tinnitus/hyperacusis severity. All patients were medically cleared of underlying pathology, which could cause these symptoms. 60.0% of the total sample (345 patients), 40.6% of tinnitus only patients, 81.1% of hyperacusis patients had ≥ 1 symptoms (P < 0.001). 68% of severe tinnitus patients, 91.3% of severe hyperacusis patients had ≥ 1 symptoms (P < 0.001). 19.7% (68/345) of patients in the total sample had AS. 83.8% of AS patients had hyperacusis, 41.2% of non-AS patients had hyperacusis (P < 0.001). The high prevalence of TTTS symptoms suggests they readily develop in tinnitus patients, more particularly with hyperacusis. Along with AS, they should be routinely investigated in history-taking.

Tinnitus pitch, masking, and the effectiveness of hearing aids for tinnitus therapy.

OBJECTIVE: To assess the benefits of hearing aids on tinnitus according to the tinnitus reaction questionnaire (TRQ; Wilson et al, 1991), to verify whether the degree of masking provided by the hearing aid influenced the TRQ score, to examine whether the matched tinnitus pitch predicted the effectiveness of hearing aids in masking tinnitus, and to determine whether prescription of high-frequency amplification might be desirable in tinnitus management when tinnitus pitch is high. DESIGN AND STUDY SAMPLE: A retrospective evaluation of the clinical outcomes of 70 tinnitus patients fitted with hearing aids was undertaken. The primary outcome measure was the TRQ, with a secondary subjective measure of tinnitus masking. RESULTS: Participants who achieved masking with their hearing aids had greater reduction in TRQ scores. Masking was more likely to be achieved when participants had good low-frequency hearing and tinnitus pitch fell into the frequency range of the hearing aids. CONCLUSIONS: The results support the use of hearing aids for tinnitus management, and suggest that masking may be a significant contributor to hearing aid success, implying that high-frequency amplification may be effective in high-pitch tinnitus.

Short-term hearing fluctuation in Meniere's disease.

This study aimed to assess the extent and implications of short-term hearing fluctuation in Meniere's disease. Thirty-six subjects diagnosed with Meniere's were recruited to measure their own hearing using in-situ audiometry via a hearing aid (Widex Diva) and a portable programmer (SP3). Self-hearing tests measuring up to 14 frequency bands were conducted three times a day over eight weeks using the expanded Sensogram. Twenty-three ears showed low frequency fluctuation while ten fluctuated in mid frequencies with some 'double peak' audiogram configurations. Eight ears in the later stages of Meniere's, contrary to expected, also recorded fluctuation across all frequencies. Self-hearing testing Meniere's ears over eight weeks revealed great hearing fluctuation with significant changes in audiogram configuration. It suggests that as endolymphatic hydrops progresses through the cochlea, low frequency fluctuation is followed by fluctuation in the mid frequencies, leading to fluctuation across all frequencies. Use of a self-hearing test may facilitate diagnosis and hearing aid fitting for this population, as clinical audiograms may not provide accurate information of hearing fluctuation.

Naunton's Masking Dilemma Revisited.

BACKGROUND: Pure-tone audiometry is essential in diagnosing clinical hearing loss. Masking of the nontest ear is mandatory for determining accurate hearing thresholds in the presence of asymmetrical levels between the two ears and for ascertaining the presence of a conductive hearing loss. Paradoxically, over masking occurs when the intensity of the required masking noise to the contralateral ear is such that it exceeds interaural cranial attenuation by an amount sufficient to mask the test ear. Ralph F Naunton was the first to describe this phenomenon, which has since been known as "Naunton's masking dilemma." METHODS: A formula was derived mathematically to predict when Naunton's masking dilemma might occur in air and bone conduction. Review of Ralph F Naunton's primary works and related publications was performed. RESULTS: Our derived mathematical formulae predict when "Naunton's masking dilemma" may occur. During air conduction testing, a masking dilemma may occur when the sum of the air/bone gaps is greater than or equal to twice the interaural attenuation minus 15 dB (Σ ABGNTE+TE ≥ 2 × IA - 15 dB). During bone conduction testing, a masking dilemma may occur when the air-bone gap of the nontest ear is greater than or equal to the interaural attenuation minus 15 dB (ABGNTE ≥ IAAIR - 15 dB). CONCLUSION: Naunton's masking dilemma imposes a significant limitation to conventional audiometric testing. To the best of our knowledge, we think this is the first time that Naunton's masking dilemma has been represented in a simplified mathematical equation.

Hearing aids for Ménière's syndrome: implications of hearing fluctuation.

BACKGROUND: Hearing fluctuation imposes the biggest challenge in the fitting of hearing aids for patients with Ménière's syndrome. PURPOSE: This study shows that the problem maybe be overcome by allowing the patients to test their own hearing and to program their own hearing aids to adjust for hearing fluctuation. RESEARCH DESIGN AND STUDY SAMPLE: A group of 40 participants diagnosed with Ménère's syndrome were fitted with Widex Senso Diva hearing aids and were provided with a portable Senso Programmer 3 (SP3) that allowed them to measure their own hearing thresholds at up to 14 different frequencies and to program their own devices. INTERVENTION: The participants were instructed to test their hearing three times a day for 8 weeks and to program their hearing aids according to the measured thresholds. DATA COLLECTION AND ANALYSIS: All participants recorded some degree of hearing fluctuation during the 8-week trial. RESULTS AND CONCLUSIONS: Among participants, 70 percent continued to program their hearing aids on a regular basis and reported great satisfaction with amplification because they are now able to adjust their own devices when their hearing fluctuates.

Cortical auditory evoked responses from an implanted ear after 50 years of profound unilateral deafness.

A male with unilateral deafness in the right ear since 8 years of age developed a sudden hearing loss in the left ear at age 63. A hearing aid was fitted in the left ear with limited benefit. The right ear received a cochlear implant (CI) 20 months later. Cortical auditory evoked potentials (CAEPs) and speech recognition scores (SRS) were measured in free-field three, six and nine months after implantation with the hearing aid alone, CI alone and bimodal condition (hearing aid and CI together). Three months after implantation the cortical responses for the two ears were similar, despite more than 50 years of unilateral auditory deprivation. CAEPs measured over time show evidence of binaural interaction and improvements in SRS.

Cochlear Implantation in Ménière's Disease With and Without Labyrinthectomy.

OBJECTIVE: To investigate outcomes of cochlear implantation (CI) in patients with Ménière's disease (MD) with and without surgical labyrinthectomy. STUDY DESIGN: Retrospective study. SETTING: Multiple tertiary referral centers. SUBJECTS: Thirty one ears from 27 patients (17 men, 10 women, aged 42-84) with CI in ipsilateral MD ear. INTERVENTION: CI in ears with intact labyrinths (Group 1), CI with simultaneous surgical labyrinthectomy (Group 2), and CI sequential to surgical labyrinthectomy (Group 3). MAIN OUTCOME MEASURE: Within-subject improvement on Bamford Kowal Bench test or City University of New York open set sentence tests. RESULTS: Majority of ears achieved excellent open-set speech recognition by 12 months post-CI, irrespective of intervention group. Preoperative details including patient age and sex, implant, MD and previous intervention, and audiological test results did not significantly affect outcomes. Patients with MD undergoing CI only may experience vestibular dysfunction which may cause long-term concerns. Incidental finding was noted of eight ears with fluctuating symptoms in ipsilateral ear during 12-month period post-CI, with five of eight ears showing objective fluctuating impedances and mapping. CONCLUSION: CI in MD can yield good hearing outcomes in all three groups and this is possible even after a long delay after labyrinthectomy. Bilateral MD patients are complex and prospective quality of life (QoL) measures would be beneficial in being better able to manage the vestibular outcomes as well as the audiological ones.

Cochlear Implant Impedance Fluctuation in Ménière's Disease: A Case Study.

OBJECTIVE: To contribute to the understanding of hearing fluctuation in Ménière's disease (MD) by disseminating a case study of a cochlear implanted ear with ongoing fluctuation of electrode impedances with episodic tinnitus and no associated vestibular symptoms. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral audiology clinic. PATIENT: Man, born in 1936, with a total hearing loss in the right ear because of Mumps at age 8 years and a fluctuating progressive hearing loss in the left ear because of Ménière's disease since age 63 years. INTERVENTION: Sequential bilateral cochlear implantation right ear in August 2002 and left ear in March 2006. MAIN OUTCOME MEASURE: Impedance measurements of implanted intracochlear electrodes via common ground stimulation using proprietor programming software. RESULTS: Electrode impedances in the MD showed significant ongoing variation since implantation, whereas the contralateral non-MD ear remained stable over a period of 9 years. CONCLUSION: Electrode impedances in the ear with MD showed a variation pattern similar to that found in the hearing fluctuation characteristic of the disease. These findings raise the possibility that the same physiological mechanisms of hearing fluctuation may be responsible for intracochlear electrode impedance changes. We hypothesize that impedance fluctuation is because of changes in the permeability of the blood-labyrinth barrier because of cyclic immune activity in the inner ear which alters the electrical resistance between scala tympani and blood.

Conventional behind-the-ear hearing aids after subtotal petrosectomy with blind sac closure.

OBJECTIVE: To overcome the limitations of monaural hearing resulting from a subtotal petrosectomy and blind sac closure by fitting of a behind-the-ear hearing aid. STUDY DESIGN: Three patients were fitted with aids. Patient satisfaction and the decision to buy the aids were recorded. RESULTS: Three patients reported good hearing result despite the absence of the middle ear structures. CONCLUSION: For patients troubled by the limitations imposed on hearing with a unilateral conductive loss following a subtotal petrosectomy and blind sac closure, a behind-the-ear aid is a simple and easy option to try, if the residual canal allows fitting of the aid.

Improving sound localization after cochlear implantation and auditory training for the management of single-sided deafness.

OBJECTIVE: To report a successful case of cochlear implantation and auditory training for the improvement of sound localization in a patient with single-sided deafness. STUDY DESIGN: Case report and literature review. SETTING: Tertiary referral otology practice. PATIENT: Fifty-seven-year-old man receiving cochlear implantation after 8 years of unilateral sensorineural hearing loss. INTERVENTION: Initially, CROS hearing aid, then osseointegrated bone conduction system and finally cochlear implantation and auditory training. MAIN OUTCOME MEASURES: Sound localization tests. RESULT: Sound localization tests after CI and auditory training showed improvement when compared with testing performed after fitting of an osseointegrated bone conduction system. CONCLUSION: Cochlear implantation followed by 3 months of auditory training may have improved sound localization in this patient with single-sided deafness. Further case-controlled studies need to be undertaken to ascertain whether CI alone without formal auditory training will promote the same results.

Behçet's disease presenting as a peripheral vestibulopathy.

Prolonged acute spontaneous vertigo can be secondary to acute vestibular neuritis or posterior circulation ischaemia. We present a 66-year-old man who first developed an acute vestibular syndrome with profound unilateral hearing loss 34 years ago. First treated as vestibular neuritis, he subsequently developed manifestations of Behçet's disease, including mouth ulcers, genital ulcers and erythema nodosum over a period of 10 years. Subsequently, sudden sensorineural hearing loss affecting his only hearing ear responded to immunomodulation, confirming an autoimmune cause for the audiovestibular symptoms. This report serves as a reminder that vestibular neuritis seldom causes hearing loss; ischaemic, infective and autoimmune causes should be sought when an acute vestibular syndrome is accompanied by hearing impairment.

Bilateral cochlear implants in long-term and short-term deafness.

This case is of a 70-year-old man with single-sided deafness (SSD) in the right ear since childhood, who developed a sudden severe hearing loss in the left ear at the age of 63. Eventually, after he received cochlear implants in both ears, he started to present behavioural auditory processing skills associated with binaural hearing, such as improved ability to understand speech in the presence of background noise, and sound localization. Outcomes were measured using cortical auditory evoked potentials, speech perception in noise, sound localization tests, and a self-rating questionnaire. The results suggest that even after more than 50 years of unilateral deafness it was possible to develop binaural interaction and sound localization as a result of electric auditory stimulation.

Are cortical auditory evoked potentials useful in the clinical assessment of adults with cochlear implants?

Cochlear implant (CI) trends are changing as more recipients are receiving bilateral implantation. Also more pre-lingually deafened adults are choosing to be implanted. Clinical assessment after cochlear implantation is usually based on speech perception tests. Such tests, however, may not be a realistic outcome measure for some of these cases, creating a need for more objective measures of CI performance. Cortical auditory evoked potentials (CAEPs) recorded in the sound field may be a fast and reliable procedure for the clinical audiologist to determine CI outcomes. This paper presents two case studies illustrating CAEP findings in an adult CI user who was pre-lingually deafened and a bilateral CI user.

Changes in audiometric thresholds before, during and after attacks of vertigo associated with Meniere's syndrome.

CONCLUSION: No significant changes in hearing thresholds were observed during vertigo attacks associated with Meniere's disease. OBJECTIVES: To determine if the hearing alters during the period of the attacks of vertigo in Meniere's disease. PATIENTS AND METHODS: The study group consisted of patients who had a clinical diagnosis of definite Meniere's syndrome according to the AAOOHNS criteria, a score on the Gibson scale of 7 or over and an enhanced negative summating potential on transtympanic electrocochleography. These patients were supplied with a programmable hearing aid and a portable programmer that allowed them to measure their own hearing in situ. They were asked to measure their audiometric thresholds daily and if possible during the attacks of vertigo. RESULTS: Six of the patients were able to measure their hearing during attacks of vertigo and their hearing thresholds obtained before, during and after the vertigo attacks were compared. Five of six subjects showed <10 dBHL change in the hearing levels at all tested audiometric frequencies before, during and after the attacks of vertigo. One subject had a probable change in threshold before the attack but not during the attack of vertigo.