Steroid Efficacy on Audiologic Recovery in Patients With Sudden Sensorineural Hearing Loss and Vestibular Schwannoma: A Retrospective Review.

OBJECTIVES: The objectives of this study are to (i) estimate the incidence of vestibular schwannoma (VS) among patients in an integrated healthcare system who present for evaluation of sudden sensorineural hearing loss (SSNHL) and (ii) evaluate the efficacy of empiric steroid therapy on audiologic recovery among SSNHL patients ultimately diagnosed with VS. METHODS: A retrospective chart review was performed on patients presenting with SSNHL in 2021 at a multicenter integrated healthcare system serving over 4 million members. Patient demographics, audiometric data, VS diagnosis, therapeutic steroid intervention, and data regarding treatment response were recorded. A clinically significant audiometric improvement was defined as (i) an increase of 15% in word recognition score, (ii) a decrease of 15 dB in four-frequency pure-tone average (PTA) using frequencies of 500, 1000, 2000, and 4000 Hz, or (iii) a PTA of <20 dB on follow-up audiogram. RESULTS: Six hundred fifty-eight patients were reviewed, of which 309 (56.0% male; mean, 57.5 years) met the inclusion criteria with audiometric data and magnetic resonance imaging data. Ten patients (70.0% male; mean, 51.3 years) were found to have VS. Of these, five patients received oral steroid therapy alone, and five had combination therapy (oral + intratympanic steroid injections). No patients received intratympanic steroid therapy alone. Median PTA improvement with steroid therapy was 3.1-dB hearing loss, and median word recognition score improvement was 16.5%. Six of 10 patients demonstrated clinically significant audiometric improvement with steroid therapy. CONCLUSION: This study represents the largest US-based study showcasing the prevalence of VS in patients originally presenting with SSNHL. It also reinforces previous findings that VS does not preclude trials of steroid therapy.

Prophylactic nimodipine treatment and improvement in hearing outcome after vestibular schwannoma surgery: a combined analysis of a randomized, multicenter, Phase III trial and its pilot study.

OBJECTIVE In clinical routines, neuroprotective strategies in neurosurgical interventions are still missing. A pilot study (n = 30) and an analogously performed Phase III trial (n = 112) pointed to a beneficial effect of prophylactic nimodipine and hydroxyethyl starch (HES) in vestibular schwannoma (VS) surgery. Considering the small sample size, the data from both studies were pooled. METHODS The patients in both investigator-initiated studies were assigned to 2 groups. The treatment group (n = 70) received parenteral nimodipine (1-2 mg/hour) and HES (hematocrit 30%-35%) from the day before surgery until the 7th postoperative day. The control group (n = 72) was not treated prophylactically. Facial and cochlear nerve functions were documented preoperatively, during the inpatient care, and 1 year after surgery. RESULTS Pooled raw data were analyzed retrospectively. Intent-to-treat analysis revealed a significantly lower risk for hearing loss (Class D) 12 months after surgery in the treatment group compared with the control group (OR 0.46, 95% CI 0.22-0.97; p = 0.04). After exclusion of patients with preoperative Class D hearing, this effect was more pronounced (OR 0.38, 95% CI 0.17-0.83; p = 0.016). Logistic regression analysis adjusted for tumor size showed a 4 times lower risk for hearing loss in the treatment group compared with the control group (OR 0.25, 95% CI 0.09-0.63; p = 0.003). Facial nerve function was not significantly improved with treatment. Apart from dose-dependent hypotension (p < 0.001), the study medication was well tolerated. CONCLUSIONS Prophylactic nimodipine is safe and may be recommended in VS surgery to preserve hearing. Prophylactic neuroprotective treatment in surgeries in which nerves are at risk seems to be a novel and promising concept. Clinical trial registration no.: DRKS 00000328 ( https://drks-neu.uniklinik-freiburg.de/drks_web/ ).

Evidence-based practice: management of adult sensorineural hearing loss.

Sensorineural hearing loss is a complex disease state influenced by genetics, age, noise, and many other factors. This article reviews our current knowledge regarding the causes of sensorineural hearing loss and reviews the more challenging clinical presentations of sensorineural hearing loss. We have reviewed the latest medical literature in an attempt to provide an evidence-based strategy for the assessment and management of sudden sensorineural hearing loss, rapidly progressive sensorineural hearing loss, and asymmetric/unilateral sensorineural hearing loss.

Contralateral hearing disturbance following posterior fossa surgery.

A 53-year-old man suffered contralateral hearing disturbance one day after acoustic neuroma surgery. Hearing function gradually recovered after steroid and hyperbaric therapy. Contralateral hearing disturbance after acoustic neuroma surgery is an extremely rare complication that can also occur after other posterior fossa surgery. The mechanism of this rare phenomenon remains unclear, but the patent cochlear aqueduct may be involved.

Tinnitus loudness in quiet and noise after resection of vestibular schwannoma.

OBJECTIVES: (1) To use a loudness model to assess the influence of loudness recruitment on estimates of the loudness of tinnitus obtained by loudness matching; (2) To compare the effect of background noise on the loudness of tinnitus for individuals who are unilaterally deaf after resection of vestibular schwannoma (VS) and those with idiopathic tinnitus. BACKGROUND: After translabyrinthine resection of VS, patients experience unilateral deafness and tinnitus in the operated ear. Most complain that their tinnitus is more bothersome in noisy environments, unlike those with idiopathic tinnitus. PARTICIPANTS: Unilaterally deaf individuals experiencing tinnitus as a consequence of VS surgery and a comparison group with idiopathic tinnitus. METHODS: Participants adjusted the level of a probe tone at the frequency where their hearing was best to match the loudness of their tinnitus in quiet; for VS participants, matches were made using a probe in the unaffected ear. Matches were then obtained in the presence of threshold-equalizing noise. RESULTS: For those with idiopathic tinnitus, the probe loudness level, calculated using a loudness model, was almost invariant with hearing loss at the probe frequency and was usually between 20 and 50 phons. For the VS group, the probe loudness level ranged from 6 to 51 phons. With increasing threshold-equalizing-noise level, the loudness match decreased slightly for the comparison group but increased significantly for the VS group. CONCLUSION: The tinnitus in quiet had a moderate loudness for both groups. Background noise slightly decreased tinnitus loudness for most participants with idiopathic tinnitus but increased tinnitus loudness for VS participants. We propose 2 possible mechanisms for the effect of noise in the VS group.

The prognostic value of speech recognition scores at diagnosis of vestibular schwannoma.

We aimed to assess whether speech recognition scores (SRS) are predictive of outcomes in patients with small vestibular schwannoma (VS) undergoing observation. Ninety-five patients with VS whose initial management was observation with serial imaging were retrospectively analysed. Patients were divided into groups according to their average hearing level and SRS at diagnosis. About 60% of patients had good initial SRS (GIS) and 40% had poor initial SRS (PIS). Mean follow-up was 44 months, during which time data were collected regarding hearing level, tumour growth and the eventual management option (continued or failed observation). Observation was discontinued by 24% (23/95) of patients. GIS-patients were more likely to maintain stable hearing than those with PIS (p<0.05). Hearing was stable in 73% (64/87) of patients. These findings indicate that patients with PIS are more vulnerable to progressive hearing loss than those with GIS. Observation may be a suitable management option for all patients with small VS, particularly those with GIS.

Vestibular schwannoma: how much residual hearing is useful?

OBJECTIVE: The objective of this study was to determine the usefulness of residual hearing in the tumour ear in vestibular schwannoma patients. DESIGN: A prospective case series study. SETTING: The study was performed at the Queen Elizabeth Health Sciences Centre in Halifax, Nova Scotia. METHODS: Thirty-three vestibular schwannoma patients and 13 controls underwent QuickSIN (Etymotic Research Inc, Elk Grove, IL) speech-in-noise testing with the tumour and good ears open and occluded. Nine testing conditions used three speakers with speech signal from the tumour side, the front, and the good ear side, with noise in other speakers. MAIN OUTCOME MEASURES: Tumour ear contribution (TEC) was calculated by the decrease in score with the index ear occluded. Multiple regression analysis and correlation coefficients were used to determine predictors of TEC. RESULTS: The strongest correlation was between the pure-tone average (PTA) and the TEC with signal from the tumour side. The speech discrimination score (SDS) was also significantly correlated with TEC in this condition. Neither PTA nor SDS correlated well with TEC with signal from other directions. Multiple regression analysis with TEC and sound from the tumour ear as the dependent variable showed that the SDS and PTA of the tumour ear are significant independent predictors (p = .049 and .037, respectively). There are no obvious breakpoints in the PTA or SDS to make 50%, 50 dB, or other operating points "special." CONCLUSION: The main contribution of residual hearing is in signals from the tumour side. The various rules are more or less equivalent in discriminating between those who will have a high TEC and those who will not.

[Vestibular evoked muscle potentials dependency on neural origin and the location of an acoustic neuroma]. / Die vestibulär evozierten Muskelpotenziale in Abhängigkeit vom nervalen Ursprung und der Lage eines Akustikusneurinoms.

INTRODUCTION: Most acoustic neuromas (AN) originate from the inferior vestibular nerve (IVN). Vestibular evoked myogenic potentials (VEMP) are accepted as the only unilateral test for the function of the sacculus and the IVN. METHODS: The influence of the origin from the IVN and superior vestibular nerve (SVN), and the position of the AN in relation to the internal auditory canal on VEMPs was investigated. A total of 39 patients (aged: 30-67 years, mean: 53 years) were examined. The VEMPs were recorded on the activated sternocleidomastoid muscle and averaged over 200 stimuli. Tone bursts (95 dB nHL; 500 Hz; stimulation rate 5 Hz) were used to generate the VEMPs. RESULTS: The exact origin of the AN from the SVN or the IVN could be determined intraoperatively and correlated using VEMP in 28 patients. CONCLUSION: The origin of the AN has only a marginal influence on the results of VEMP measurements. The position of the AN in relation to the internal auditory canal seems to have more influence than the origin.

Delayed facial nerve paresis following acoustic neuroma resection and postoperative vasoactive treatment.

OBJECT: Delayed facial nerve paresis is a well known clinical phenomenon following acoustic neuroma surgery, typically occurring early during the postoperative course. The clinical course of the delayed facial nerve paresis and intraoperative electromyographic (EMG) signals were evaluated in a subgroup of patients who underwent vasoactive treatment for preservation of hearing and developed secondary deterioration after termination of treatment. METHODS: Between 1990 and 2001 seven patients were identified who received vasoactive treatment for preservation of hearing and developed a delayed facial nerve paresis after termination of medication. Intraoperative facial nerve EMG activity was analyzed in six patients. RESULTS: All patients developed a delayed facial nerve paresis between 2-5 days following termination of a 10 day treatment consisting of HES and nimodipine. Medication was re-initiated and the facial nerve paresis improved in all patients. In two patients intraoperative EMG signals revealed "A-trains" waveform patterns, which are highly suggestive for an immediate postoperative facial nerve paresis, whereas in four patients no pathognomonic EMG patterns could be recorded. CONCLUSIONS: The delayed onset of a facial paresis following termination of vasoactive treatment points to a disturbed microcirculation of the nerve as the main pathophysiological feature. Two groups could be identified on the basis of intraoperative EMG activity. In one group with presence of "A-trains" medication apparently masked the onset of an immediate postoperative facial nerve deficit. Four patients without "A-trains" did not develop a typical delayed facial nerve paresis during vasoactive treatment, but thereafter. The time lag between termination of treatment and onset of a delayed palsy points to a protective effect due to improved microcirculation.

Transcranial contralateral cochlear stimulation in unilateral deafness.

OBJECTIVES: The purpose of this study is to evaluate the effectiveness of Bone Anchored Cochlear Stimulator (BAHA) in transcranial routing of signal by implanting the deaf ear. Study design and settings Eighteen patients with unilateral deafness were included in a multisite study. They had a 1-month pre-implantation trial with a contralateral routing of signal (CROS) hearing aid. Their performance with BAHA was compared with the CROS device using speech reception thresholds, speech recognition performance in noise, and the Abbreviated Profile Hearing Benefit and Single Sided Deafness questionnaires. RESULTS: Patients reported a significant improvement in speech intelligibility in noise and greater benefit from BAHA compared with CROS hearing aids. Patients were satisfied with the device and its impact on their quality of life. No major complications were reported. Conclusion and significance BAHA is effective in unilateral deafness. Auditory stimuli from the deaf side can be transmitted to the good ear, avoiding the limitations inherent in CROS amplification.

[Idiopathic sudden deafness]. / Les surdités brusques idiopathiques.

PURPOSE: Sudden idiopathic deafness is a sensorineural hearing loss with no recognized causes at the time of onset. The impairment site is usually localized in the cochlea, but some cases of retrocochlear lesions (e.g., cerebellopontine angle tumors, degenerative neural diseases, neuraxial ischemic lesions) can induce sensorineural deafness. The medical management of patients presenting with sudden deafness aims at detecting a causal mechanism, and at administering emergency therapeutic drugs. The diagnosis of idiopathic sudden deafness can be definitely made when no causes are found. Usually, the impairing mechanism involves the cochlea. The pathophysiology of this sensorineural alteration is still unknown. It is most likely that several mechanisms are associated together, their common point being an impairment to the feedback loop of the organ of Corti. CURRENT KNOWLEDGE AND KEY POINTS: It is very likely that reactivation of neurotropic viruses and/or cochlear ischemia are frequent etiologies. Whatever the cause, the treatment is to be administered urgently, and consists of a high-dose corticotherapy at the least. Other treatments have never really proven to be effective. It is secondarily checked that no retrocochlear pathological processes, such as a cerebellopontine angle tumor, is present, in particular in young people. FUTURE PROSPECTS AND PROJECTS: One of the current objectives is to determine when cochlear ischemia is involved, in a mini-invasive manner, such as with laser Doppler flowmetry, so that the treatment can be optimized. From a therapeutic point of view, early acoustic protection has been proven to be effective in cases of cochlear ischemia in small laboratory animals. Its efficacy in case of sudden deafness, non-exclusive of other causes than ischemia, is being assessed in a multicentric project.

A comparison of inter-aural attenuation with the Etymotic ER-3A insert earphone and the Telephonics TDH-39 supra-aural earphone.

One of the many reported advantages of the insert earphone over the supra-aural earphone is increased inter-aural attenuation (IA). Minimum values of IA determine the need for masking of the non-test ear in air-conduction audiometry. The aim of the present study was to measure inter-aural attenuation for the Etymotic Research ER-3A insert earphone (with deep and shallow insertion of the ear plug within the ear canal) and compare this with the supra-aural Telephonics TDH-39/MX41-AR earphone/cushion combination. Subjects were 18 adults ranging in age from 38 to 68 years (mean 50 years). Each subject had no hearing in one ear following translabyrinthine surgery for removal of an acoustic neuroma. The opposite ear had hearing thresholds better than 40 dB HL and an air-bone gap of less than 10 dB at any audiometric frequency. Pure tone air-conduction thresholds were obtained in the range 0.25-8 kHz. Deep insertion of the insert earphone was deemed to occur when the outside edge of the ear plug was flush with the entrance of the ear canal. Shallow insertion was deemed to occur when half of the ear plug (6 mm) was inside the entrance of the ear canal. IA was defined operationally as the difference between the good-ear and poor-ear not-masked air conduction threshold for a given audiometric frequency and earphone. The results show that the TDH-39/MX41-AR combination provides a median IA of approximately 60 dB with a lower limit of approximately 45 dB. Greater IA was obtained with the ER-3A insert earphone but this depended on the depth of insertion. With a deep insertion, the 1A values were some 15-20 dB greater than with the supra-aural earphone. Although frequency-specific IA values are provided, a simple rule of thumb is to apply masking to the non-test ear when the pure tone airconduction signal from the ER-3A insert earphone exceeds the bone conduction threshold of the non-test ear by 55 dB HL or more. If it is not possible to obtain a deep insertion depth this value should be reduced by 5 dB.

Tinnitus program at Brasília University Medical School.

Over the last 6 months, all patients seen at the otologic clinic of Brasília University Medical School answered a questionnaire designed to identify and describe the symptom of tinnitus. A total of 500 patients reported and described this symptom. They underwent physical examination, laboratory tests and audiological evaluation. In their order of frequency, presbycusis, chronic otitis media, otosclerosis, acoustic trauma, Menière's disease, ototoxicity, and vestibular schwannoma were found. Tinnitus was rated as minor in 81%, moderate in 18%, and severely disabling in 1%. Those who requested treatment for tinnitus were treated medically. Central vasodilators, vestibular suppressants, calcium channel blockers, anticholinergic drugs, anticonvulsant drugs, and gingko biloba were used with variable results. Tinnitus maskers were not used, but hearing prostheses were fitted when indicated. Treatment failed in the 1% with severe disabling tinnitus, and they were entered in a double-blind, randomized protocol for intratympanic dexamethasone injection. Under topical anesthesia, 0.2 ml of a 4-mg/ml dexamethasone solution (0.8 mg per injection) or 0.2 ml of normal saline was injected just posterior to the umbo. Patients remained supine for 20 minutes with the injected ear up and received four injections at 1-week intervals. Preliminary results are reported. Tinnitus is a very frequent symptom among our otologic patients, but most of them would not mention the symptom spontaneously, probably because for 81% it was mild. Curiously, the 5% of the severely disabling type tend to exhibit no clear cause, whereas the mild and moderate cases usually have an identifiable etiology.

Improvement of action tremor by chronic cortical stimulation.

The authors present a patient who had long-term improvement of a severe upper limb action tremor after chronic cortical stimulation. A 40-year-old woman complained of facial pain and tremor of the left arm after removal of an acoustic neurinoma. A motor cortex stimulation was performed to treat the deafferentation facial pain in 1993. Chronic cortical stimulation induced complete relief of both pain and tremor and allowed the patient to recover functional capacity of the limb. These effects persisted throughout a 32-month follow up. Differential effects on pain and tremor were observed when parameters of stimulation were varied, suggesting different mechanisms for the relief of pain and tremor. Attention was focused on control of the tremor. This effect could be the result of the inhibition of subcortical structures which are involved in tremor. Chronic cortical stimulation appears to be an effective treatment for controlling severe action tremors.

Rehabilitación oculopalpebral post-parálisis facial periférica por sección completa del tronco: colocación de implante de oro y kinesioterapia / Treatment of complete facial paralysis with early gold weight lid implant

La tarsorrafia fue el método alternativo para el tratamiento de las complicaciones post-lesión completa del nervio facial. El implante de una lámina de oro en el párpado permite obtener una solución funcional y cosmética a la falta de oclusión palpebral. Cuando la lesión lleva mucho tiempo de evolución, se suele agregar ectropion de párpado inferior que puede ser resuelto con la técnica de Kuhnt-Szymanovsky en el mismo momento quirúrgico en que se coloca el implante. En nuestra experiencia de 10 pacientes tratados desde Marzo del '93 a Diciembre del '96, hemos colocado en 9 de ellos el injerto de oro, en un caso corregido el ectropion quirúrgicamente y en otro solo se realizó kinesioterapia de rehabilitación (por negativa de la paciente a colocarse el implante quirúrgicamente). En todos realizamos kinesioterapia de rehabilitación precoz. Igual que otros autores, este es un método sencillo y eficaz, alternativo a la tarsorrafia

Rehabilitación oculopalpebral post-parálisis facial periférica por sección completa del tronco: colocación de implante de oro y kinesioterapia / Treatment of complete facial paralysis with early gold weight lid implant

La tarsorrafia fue el método alternativo para el tratamiento de las complicaciones post-lesión completa del nervio facial. El implante de una lámina de oro en el párpado permite obtener una solución funcional y cosmética a la falta de oclusión palpebral. Cuando la lesión lleva mucho tiempo de evolución, se suele agregar ectropion de párpado inferior que puede ser resuelto con la técnica de Kuhnt-Szymanovsky en el mismo momento quirúrgico en que se coloca el implante. En nuestra experiencia de 10 pacientes tratados desde Marzo del 93 a Diciembre del 96, hemos colocado en 9 de ellos el injerto de oro, en un caso corregido el ectropion quirúrgicamente y en otro solo se realizó kinesioterapia de rehabilitación (por negativa de la paciente a colocarse el implante quirúrgicamente). En todos realizamos kinesioterapia de rehabilitación precoz. Igual que otros autores, este es un método sencillo y eficaz, alternativo a la tarsorrafia (AU)

Mechanisms of hearing loss in acoustic neuroma: an otoacoustic emission study.

Evoked otoacoustic emissions (EOAE) are active mechanical responses from the cochlea which provide information about the integrity of the preneural cochlear receptor mechanisms. It may be hypothesised, therefore, that if a hearing impairment is neural in origin, normal EOAEs may be obtained from the cochlea, which, although dissociated, is functioning normally. This study examined the status of the cochlea with EOAE in patients with cochlear (Meniere's disease) and neural (surgically proven acoustic neuroma) disease. In patients with presumed cochlear lesions, no emissions were present with mean hearing worse than 40 dB across a frequency range of 0.5 to 4 kHz. Similarly, an EOAE was not present in any of the 26 acoustic neuroma patients studied when the average (0.5 to 4 kHz) hearing was greater than 40 dB. We conclude that dissociation of the cochlea in patients with acoustic neuroma appears to be rare and, in fact, cochlear involvement occurs in most cases. Possible mechanisms responsible for the effect on the cochlea in this group include degenerative changes due to chronic partial obstruction of the blood supply by the tumour, biochemical alterations in the inner ear fluids, loss of efferent control of active mechanical tuning, and hair cell degeneration secondary to neuronal loss in the eighth nerve.

Frequency selectivity in patients with acoustic neuroma.

Frequency selectivity was compared in subjects with hearing loss due to acoustic neuroma and cochlear pathology, and normal listeners. A particular interest was the role of probe tone parameters on the shape of the tuning curve. Psychophysical tuning curves (PTCs) were measured for each of two equal energy 2000-Hz probe tones (10 dB SL/300 msec and 17 dB SL/60 msec), using simultaneous 1/3-octave narrow-band noise maskers centered at 1, 1.25, 1.6, 2.5, 3.15, and 4 kHz. The results showed that the critical masker levels obtained for impaired listeners were significantly greater than those from normal subjects. The slope of the low-frequency limb of the PTC was steeper for normal compared to hearing-impaired listeners but there was no difference due to site of lesion. In all three groups, the critical masker levels obtained with the short probe were significantly greater than those for the long probe, negating the hypothesis that equal energy probes would yield the same outcomes. Tuning in listeners with hearing loss was highly correlated with audiometric threshold but not with tumor size, width of the internal auditory canal, or tumor location within the cerebellopontine angle. The main conclusion was that cochlear and retrocochlear hearing loss are similar with respect to their effect on frequency selectivity.

Tinnitus suppression by electrical promontory stimulation in sensorineural deaf patients.

In 62 patients, whose hearing acuity was normal or profoundly impaired, tinnitus suppression was attempted through electrical promontory stimulation (EPS) with a Promontory Stimulator (Cochlear Co.). The causes of tinnitus were idiopathic sudden deafness, ototoxicity, noise induced hearing loss, labyrinthitis, Meniere's disease, acoustic neuroma and unknown origin. Reduction of tinnitus was achieved in 46 of 68 ears (67.6%). Of these, EPS was most effective in cases of noise induced hearing loss (100.0%; 3/3), followed by those of idiopathic sudden deafness (87.5%; 14/16), Meniere's disease (83.3%; 5/6), labyrinthitis (75.0%; 6/8), ototoxicity (66.6%; 4/6) and unknown origin (76.4%; 13/17). The treatment had hardly any effect on patients with acoustic neuroma (8.3%; 1/12). Twenty patients reported residual inhibition with a duration ranging from several hours to one week. Our results suggest that tinnitus due to cochlear lesions can be suppressed by EPS. This technique may also be useful for differentiation between tinnitus resulting from cochlear lesions and from retrocochlear lesions.