Cochlear implantation in patients with Charcot-Marie-Tooth disease: two cases with a review of the literature.

PURPOSE: To report two cases of bilateral cochlear implantation (CI) in Charcot-Marie-Tooth disease (CMT) patients with novel mutations. Furthermore, we conducted a detailed literature review on the profile and outcomes of CI in this uncommon clinical circumstance. CASE PRESENTATION: Case 1 involved a 25-year-old woman who was referred for sudden hearing loss (HL) in her left ear and had a 7-year history of HL in her right ear. She was diagnosed with CMT type 1 with a thymidine phosphorylase gene mutation. CI was performed on her left side because her hearing gradually worsened to deafness in both ears. At 3 months post-operation, her speech discrimination score without lip-reading improved from 0 to 100%. She underwent a second CI on her right ear 6 months after her first CI. Two years from her first operation, the speech discrimination score was 100%. Case 2 received her first CI on her right ear at the age of nine for her bilateral HL. She was diagnosed with CMT type 2 with a Twinkle mitochondrial DNA helicase gene mutation. Preoperatively, the speech discrimination score in both ear-aided conditions was 70%. At the 7-year post-operation follow-up, the speech discrimination score was 76%. A second CI was performed due to decreasing hearing ability in her left ear. The speech discrimination score showed 100% at 7 months after the second CI. CONCLUSIONS: CI is an effective hearing rehabilitation option for CMT patients with severe-to-profound SNHL. Neuro-otologists should consider CI as a treatment option, even though hearing loss in CMT is associated with auditory neuropathy spectrum disease (ANSD).

Cochlear Implantation Following Explorative Tympanotomy in Patients With Sudden Sensorineural Hearing Loss: Surgical Features and Audiological Outcomes.

OBJECTIVE: To investigate the anatomical status of the round window niche and hearing outcome of cochlear implantation (CI) after explorative tympanotomy (ExT) with sealing of the round window membrane in patients with sudden sensorineural hearing loss at a tertiary referral medical center. METHODS: Between January 1, 2007, and July 30, 2020, 1602 patients underwent CI at our department. Out of these, all patients previously treated by ExT with sealing of the round window membrane because of unilateral sudden hearing loss were included in the study. A retrospective chart review was conducted concerning method of round window membrane sealing, intraoperative findings during CI, postoperative imaging, and hearing results. RESULTS: Twenty one patients (9 females; 8 right ears; 54.3 years [± 12.9 years]) underwent ExT with sealing of the round window membrane with subsequent CI after 26.6 months (± 32.9 mo) on average. During CI, in 76% of cases (n = 16), the round window niche was blocked by connective tissue due to the previous intervention but could be removed completely in all cases. The connective tissue itself and its removal had no detrimental effects on the round window membrane. Postoperative computed tomography scan showed no electrode dislocation. Mean postoperative word recognition score after 3 months was 57.4% (± 17.2%) and improved significantly to 73.1% (± 16.4%, P = .005) after 2 years. CONCLUSION: Performing CI after preceding ExT, connective tissue has to be expected blocking the round window niche. Remaining tissue can be removed safely and does not alter the round window membrane allowing for a proper electrode insertion. Short- and long-term hearing results are satisfactory. Consequently, ExT with sealing of the round window membrane in patients with sudden sensorineural hearing loss does not impede subsequent CI that can still be performed safely.

Sudden Sensorineural Hearing and Vestibular Loss in a Case of Relapsing Polychondritis.

OBJECTIVES: To report a case of profound bilateral sensorineural hearing and vestibular loss from relapsing polychondritis and hearing outcomes after cochlear implantation. METHODS: Case report and literature review. RESULTS: A 43 year-old woman developed sudden loss of hearing and balance that progressed over several weeks to bilateral, profound hearing and vestibular loss. Steroid treatments were ineffective. She underwent vestibular physical therapy and left cochlear implantation. About 10 months after her initial presentation, she developed erythema, warmth, swelling, and pain of the left auricle sparing the lobule, flattening of the bridge of her nose, and right ankle swelling, warmth, and skin erythema. A biopsy of the left auricle revealed histopathologic findings consistent with relapsing polychondritis. She was treated with high dose prednisolone. The ear inflammation resolved, however, despite excellent auditory response to pure tone thresholds, the patient reported no improvement in speech perception after cochlear implantation. CONCLUSIONS: Relapsing polychondritis can present with rapidly progressive, profound loss of hearing and vestibular function. Hearing outcomes after cochlear implantation can include poor speech discrimination despite good pure tone detection thresholds.

Cochlear Implantation as a Treatment for Sudden Autoimmune Sensorineural Hearing Loss in a Patient Affected by Eosinophilic Granulomatosis with Polyangiitis: A Case Report and A Review of Literature.

OBJECTIVE: To report presentation, diagnostic process, management and outcome of a case of autoimmune inner ear disease (AIED) related with Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA), treated with cochlear implantation, and review of relevant literature. CASE PRESENTATION AND MANAGEMENT: A retrospective case report of AIED associated with EGPA treated with cochlear implantation was described. A multi-step approach for diagnosis and confirmation of AIED and hearing rehabilitation was conducted, eventually leading to left cochlear implantation. RESULTS: The surgery was without complications and postoperative course was uneventful. Two years after surgery, pure-tone and speech soundfield audiometry with left cochlear implant switched on showed a good improvement in pure-tone threshold and a word recognition score of 50% at 60 dB nHL. Literature review does not report any previous case of AIED EGPA-related. CONCLUSIONS: Cochlear implantation in AIED EGPA-related have been shown to be a viable treatment option in a stabilized phase of disease.

Intratympanic steroids as a salvage therapy for severe to profound idiopathic sudden sensorineural hearing loss.

BACKGROUND: Idiopathic sudden sensorineural hearing loss (ISSNHL) is defined as a decline in hearing affecting three or more frequencies by 30 dB Objective: The aim of this study was to evaluate the results of intratympanic steroids as a salvage treatment for severe ISSNHL. MATERIALS AND METHODS: A regimen of three IT steroid injections was offered to patients who failed a 7-days intravenous steroid treatment. Eighty-four patients underwent IT salvage treatment (IT group). Their outcomes were compared with those of 255 patients with severe ISSNHL who received the same intravenous steroid regimen without salvage IT steroid therapy (Control group). RESULTS: 56% of the patients in the IT group had a hearing improvement of >15 dB after one month. The average hearing improvements were 26.5 ± 28 dB and 27.9 ± 24 dB in the IT group and the Control group, respectively (p = .67). However, patients with a type E audiogram pattern (total deafness), displayed a substantial hearing gain. CONCLUSION: Intratympanic steroids failed to show a global auditory benefit as a salvage treatment in patients with severe ISSNHL. SIGNIFICANCE: Our data suggest that a salvage treatment with intratympanic dexamethasone may be offered to patients with total deafness for whom the first systemic treatment has failed.

Computed tomography versus magnetic resonance imaging in paediatric cochlear implant assessment: a pilot study and our experience at Great Ormond Street Hospital.

BACKGROUND: To date, there is a lack of consensus regarding the use of both computed tomography and magnetic resonance imaging in the pre-operative assessment of cochlear implant candidates. METHODS: Twenty-five patients underwent high-resolution computed tomography and magnetic resonance imaging. 'Control scores' describing the expected visualisation of specific features by computed tomography and magnetic resonance imaging were established. An independent radiological review of all computed tomography and magnetic resonance imaging scan features was then compared to the control scores and the findings recorded. RESULTS: Agreement with control scores occurred in 83 per cent (20 out of 24) of computed tomography scans and 91 per cent (21 out of 23) of magnetic resonance imaging scans. Radiological abnormalities were demonstrated in 16 per cent of brain scans and 18 per cent of temporal bone investigations. CONCLUSION: Assessment in the paediatric setting constitutes a special situation given the likelihood of congenital temporal bone abnormalities and associated co-morbidities that may be relevant to surgery and prognosis following cochlear implantation. Both computed tomography and magnetic resonance imaging contribute valuable information and remain necessary in paediatric cochlear implant pre-operative assessment.

Prognostic factors regarding the hearing outcome in severe to profound sudden sensorineural hearing loss treated by tympanotomy and sealing of labyrinthine windows after ineffective systemic corticosteroid application.

PURPOSE: Tympanotomy and sealing of labyrinthine membranes has become in some centers used to treat severe to profound sudden sensorineural hearing loss refractory to conservative treatment. The aim of this retrospective study was to determine which preoperative factors influence the likelihood of postoperative recovery according to different audiological assessment criteria. METHODS: The mean final hearing threshold, the hearing improvement, the probability of a complete recovery according to two different classifications, and probability of a significant recovery of 136 adult subjects were studied by univariate and multivariate analyses. RESULTS: The subject's mean postoperative 4-pure-tone-average was 63.9 ± 35.9 dB, the mean improvement was 42.8 ± 32.6 dB. Depending on the classification system used, 18.4-28.0% of subjects experienced a complete recovery. 77.2% of subjects had a significant hearing improvement. History of a pressure change (odds ratio (OR):4.6) was the only positive prognostic factor for hearing improvement. It also enhanced probability of experiencing a complete hearing recovery (OR: 2.8-6.3). Preoperative total deafness (OR: 1.5-1.9) and vertigo (OR: 3.3-4.6) were negative prognostic factors for the mean final hearing threshold and the probability of a complete hearing recovery. Patients with a preceding pressure change event achieved a complete recovery in 45.5-50.0%, those without such an event recovered completely only in 13.2-23.7%. CONCLUSION: Evaluating prognostic factors and the rate of complete hearing recovery are influenced by the underlying assessment parameters. In addition to the mean postoperative hearing threshold and hearing gain, the probability of regaining a serviceable hearing is clinically important for the individual and should be added to the assessment criteria in future studies.

Cochlear Implantation in Postmeningitic Deafness.

PURPOSE: The aim of this study is to evaluate long-term outcomes of cochlear implantation (CI) in patients with postmeningitic deafness. METHODS: Twenty-seven patients with severe to profound hearing loss due to bacterial meningitis and received CI were the subjects of this study. Surgical findings and long-term audiological performances were evaluated. Speech perception and speech intelligibility of the implanted patients were evaluated with the categories of auditory performance-II (CAP-II) test and speech intelligibility rating (SIR) test, respectively. RESULTS: Eighteen of the 27 patients had received full electrode insertion through the patent cochlear lumen. Remaining 9 patients had varying degrees of ossification throughout the cochlea and needed to be drilled to achieve partial electrode insertion. None of the patients exhibited surgical complication. Scores in both test batteries (CAP-II and SIR) were comparable between patients who received full or partial electrode insertion (P > 0.05). CONCLUSION: Cochlear implantation after postmeningitic deafness has favorable outcomes especially in long term. Although this type of inner ear pathology may require special considerations during surgery, it is a relatively safe procedure.

The role of explorative tympanotomy in patients with sudden sensorineural hearing loss with and without perilymphatic fistula.

PURPOSE: The purpose of this study was to describe the role of explorative tympanotomy in patients with Profound Sudden Sensorineural Hearing Loss (SSNHL) without clinical evidence of perilymphatic or labyrinthine fistula and to compare intraoperative findings with the postoperative hearing outcome. STUDY DESIGN: Retrospective study of all patients diagnosed with SSNHL who underwent explorative tympanotomy between 2002 and 2005. SETTINGS: Tertiary care university-affiliated hospital. SUBJECTS AND METHODS: Eighty-two patients were diagnosed with unilateral profound SSNHL and underwent tympanotomy with sealing of the round and oval windows. Values of pure tone audiograms and percentage hearing loss of patients with and without intraoperative diagnosed perilymphatic fistula (PLF) were compared and analyzed. RESULTS: PLF was diagnosed in 28% cases intraoperatively. In most cases, hearing improved significantly after surgery. Interestingly, patients with PLF had a 2.4 times greater decrease of percentage hearing loss compared to patients without PLF. CONCLUSIONS: Explorative tympanotomy seems to be useful in patients with profound SSNHL. Patients with PLF benefit more from the surgical procedure and have better outcome than patients without PLF.

Sealing of the round and oval window niches with triamcinolone-soaked fascia as salvage surgical therapy in sudden sensorineural hearing loss.

CONCLUSIONS: Triamcinolone-soaked fascia seems to show better hearing improvement when added to tympanotomy for sudden idiopathic sensorineural hearing loss (SSHL), compared to fascia round window occlusion without triamcinolone. OBJECTIVES: To analyse if adding triamcinolone to sealing the round and oval window niches with fascia results in improved audiological outcome for acute SNHL. METHODS: Fifty-three patients (27m:43 ± 12 years, 26f:45 ± 14 years) with acute SSHL ≥50dB over 3 frequencies, who failed primary therapy, underwent transcanal tympanotomy. Twenty-five patients (Group A;cortisone:14m, 11f:46 ± 9 years) received sealing of the round and oval window with fascia soaked in triamcinolone (1ml; 40mg/ml) and 28 controls (Group B;no-cortisone:13m, 15f, 42 ± 12 years) without triamcinolone. Frequency specific and pure tone average (PTA =500-1000-2000-3000Hz) results were compared between Group A and B pre- and postoperatively. RESULTS: In Group A the PTA improved by ≥10dB in 21/25(83%) cases; in Group B 18/28(63%). Group A showed a statistically significantly better improvement across all frequencies, while linear regression revealed a significant decrease of posttherapeutic PTA to 94.96% of the initial PTA (p = .037). The overall PTA improved by 24dB. Group A improved from 73dB to 41dB(-32dB) PTA, Group B improved from 76dB to 56dB PTA (-20dB) (p < .05). Group A showed a significant additional decrease of 12.8dB (p < .001).

Predictors of hearing recovery in patients with severe sudden sensorineural hearing loss.

BACKGROUND: Sudden sensorineural hearing loss (SSHL) is a disease, which severely affects the patient's social and relational life. The underlying pathomechanisms have not been finally clarified yet and outcome is not predictable. METHODS: We conducted a retrospective study in order to identify parameters that influence hearing recovery. The data base contains results of basic otoneurological tests and clinical parameters of 198 patients with idiopathic SSHL of at least 60 dB in at least four frequencies, diagnosed and treated at the University Hospital of Münster, Germany, between 1999 and 2015. Hearing recovery was measured by pure tone audiometry. RESULTS: Multivariate linear and logistic regression analyses indicate that the chance as well as the magnitude of hearing recovery is higher for patients with normal caloric testing than for patients with pathological caloric testing. However, for the subgroup of patients who attained a hearing recovery, the caloric testing result was not found to influence the magnitude. Instead, the magnitude was noticeably lower for patients within this subgroup who had a previous hearing loss. Furthermore, we found indications that the magnitude is higher for men than for women and that receiving a high-dose steroid therapy is associated with a higher chance and magnitude of a hearing recovery. CONCLUSIONS: We conclude that SSHL associated with disorders of the vestibular system or previous hearing loss represent special sub-entities of SSHL that may be caused by unique pathophysiological mechanisms and are associated with worse outcome. Furthermore, our data support the importance of elevated dosage of steroids in SSHL therapy.

Cochlear implantation in chronic demyelinating inflammatory polyneuropathy.

OBJECTIVE: To describe a case of chronic inflammatory demyelinating polyneuropathy (CDIP) with bilateral sudden sensorineural hearing loss who subsequently benefited from unilateral cochlear implantation. METHODS: case history review and review of the literature for the terms CDIP, hearing loss, cochleovestibular dysfunction, and cochlear implantation. RESULTS: A 49-year-old woman presented with bilateral rapidly progressive sensorineural hearing loss (SNHL) 1 month after an upper respiratory tract infection. Hearing loss was not responsive to high-dose steroids and there were no other laboratory abnormalities or physical findings. Within 1 month, she developed ascending motor palsy, requiring long-term ventilator support. This neurologic condition was diagnosed as CDIP and she was successfully treated with plasmapheresis and intravenous immunoglobulin. Her hearing never recovered. At the time of cochlear implant, she had no response at the limits of the audiometer and obtained 0% on AzBio testing. No ABR could be recorded preoperatively. She underwent uneventful cochlear implantation with a perimodilar electrode. One year after activation, she had a PTA of 20 dB and 40% on AzBio sentence testing. Her eABR demonstrated a neuropathy pattern. Only two other cases of CDIP associated with dysfunction of the eighth nerve have been described, and neither had documented profound hearing loss. CONCLUSIONS: Severe SNHL associated with CDIP is rare. Although this patient has good access to sound, speech discrimination is poor at 1-year post implantation. This outcome may be due to incomplete recovery of myelination of the eighth nerve. Other possibilities include loss of peripheral nerve fibers due to the initial viral upper respiratory infection, which may lead to less neural substrate to stimulate.

Cochlear implantation in delayed sudden hearing loss after conservative vestibular schwannoma surgery.

This is a case of successful cochlear implantation in a 50-year-old man who experienced sudden hearing loss and developed ipsilateral severe tinnitus at three years following conservative stage 1 vestibular schwannoma retrosigmoid surgery. After cochlear implantation, tinnitus improved from THI grade 4 to 2. Localisation skills improved. Hearing in noise (S/N + 7 dB) with target signal from the operated side improved from 38 to 100% of correct answers. A significant improvement of spatial and speech items of the "speech, spatial and qualities of sounds" questionnaire was also measured. In conclusion, cochlear implantation is a feasible and effective solution after conservative vestibular schwannoma surgery should delayed hearing loss occur.

[Stellate ganglion catheter retention with discontinuous block on efficacy and safety in the treatment of sudden deafness].

OBJECTIVE: To investigate effect and safty evaluation of stellate ganglion catheter retention with discontinuous block on sudden deafness. METHOD: One hundred and twenty-six patiens with sudden monaural deafness were randomly divided into Catheterp and block and control groups with 42 cases in each group. All patients' throats were given conventional blood activating drugs, hormone and hyperbaric oxygen therapy. stellate ganglion puncture retained catheter were administrated to the patients in catheter group followed by ropivacaine block 1 times/day, block group stellate ganglion puncture and ropivacaine block 1 times/day. The patients in control group were only received routine comprehensive treatment. Patients in both catheter group and block groups were treated by hyperbaric oxygen therapy after the block treatment. Curative effects of three groups were observed. The patients' satisfaction, heart rate, the chages of blood pressure before and after the block, detachment of tubes, and adverse drug reaction were recorded. RESULT: The effect of the treatment in both catheter group, block group was better than in control group (85.7%, 37 cases); 83.3%, 35 cases) vs 64.3%, 27 cases, P < 0.05). The satisfactory rate in the patients in catheter group was significantly higher than block group (83.3%, 35 cases vs 61.9%, 26 cases, P < 0.05). The heart rate and the blood pressure before and 5 minutes after catheterization in catheter group and block groupwere changed obviously. Moreover, no adverse drug reaction and detachment of tubes were observed. CONCLUSION: It is a safe and effective administration of stellate ganglion catheter retention with interrupted ropivacaine block.

Cochlear implantation in unilateral sudden deafness improves tinnitus and speech comprehension: meta-analysis and systematic review.

OBJECTIVE: In recent years, otologists have begun to place cochlear implants into nonfunctioning ears after sudden unilateral hearing loss. Patients in these trials demonstrate differing degrees of hearing loss in the unimplanted ear. Few studies have examined the role of implantation in patients with normal hearing in the unimplanted ear. To understand if this practice benefits these patients in terms of tinnitus, sound localization, and speech understanding, the available world literature is reviewed. DATA SOURCES: MEDLINE, Embase, and Cochrane databases were searched for publications from database inception to June 1, 2013, without restriction of language. STUDY SELECTION: A search of multiple medical databases was performed to identify articles reporting cases series of cochlear implantation for unilateral hearing loss. Subjects were included for analysis only if the course of hearing loss was acute and rapidly progressive, if the loss was severe to profound, and if the contralateral ear had normal hearing. DATA EXTRACTION AND SYNTHESIS: Nine appropriate articles were identified, in which 36 patients met our inclusion criteria. Three meta-analyses were performed: of tinnitus (22 patients); of the lowest signal-to-noise ratio, which still allowed 50% sentence understanding (16 patients); and of sentence understanding at a fixed signal-to-noise ratio (12 patients). These found that measures of tinnitus reduction and decreased signal-to-noise ratios to still allow 50% speech discrimination were statistically significantly reduced. Systematic review of subjective changes of tinnitus in 27 patients, speech understanding in 16 patients, and sound localization in 16 patients found 96%, 100%, and 87% were improved, respectively. CONCLUSION: Cochlear implantation in unilateral sudden hearing loss with a normal functioning contralateral ear might prove to be an effective therapy. Tinnitus is reduced as is the signal-to-noise ratio, which still allows 50% speech discrimination. All patients felt that they localized sound better, and most felt that they understood speech better. Further studies should be conducted to compare the success of hearing rehabilitation of cochlear rehabilitation and traditional modalities such as contralateral routing of signal and bone-anchored hearing aids.

Auditory rehabilitation after cochlear implantation in adults with hearing impairment after head trauma.

INTRODUCTION: In this paper, the authors analyze the auditory rehabilitation after cochlear implantation in adults with hearing impairment after head trauma, comparing their performance with that of other cochlear implant (CI) adult users who have post-lingual hearing impairment with other etiologies. METHODS: The participants were divided into two groups: group 1 (N = 14) composed of CI adult users who have acquired severe to profound hearing loss after head trauma; group 2 (N = 231) composed of CI adult users who have severe to profound hearing loss from other etiologies. Performance was assessed using the following tests: tonal audiometry, speech audiometry, consonantal phonemes identification test, 100 words test, 100 words through the telephone test, monosyllables test, numbers test, sentences test, and sentences through the telephone test. RESULTS: Average results from group 1 were lower when compared with those of group 2 in all the tests used. No statistically significant difference was found for most tests. Statistically significant difference was found for consonantal phonemes identification test, 100 words through the telephone test, monosyllables test (when analyzed regarding the phonemes correctly repeated), and sentences through the telephone test. DISCUSSION: The performance of the group of CI adult users who have acquired hearing impairment after head trauma was globally lower than that observed on the group of hearing impairment with other etiologies. However, the difference was not statistically significant for most tests. Despite this difference in performance, the results from the group of CI adult users who have acquired hearing impairment after head trauma show the effectiveness of auditory rehabilitation through cochlear implantation in these situations.

Computed tomographic findings of X-linked deafness: a spectrum from child to mother, from young to old, from boy to girl, from mixed to sudden hearing loss.

PURPOSE: Congenital mixed hearing loss associated with fixed stapes footplate is a rare disorder transmitted through X-linked inheritance. The purpose of this study was to report the radiologic findings of X-linked deafness with middle ear anomalies in affected children and young patients and in carrier women. MATERIALS AND METHODS: The computed tomographic and audiometric findings of 7 subjects (4 affected children and young patients, 1 of whom is a girl; 2 carrier mothers; and a man who presented with sudden hearing loss) from different families were analyzed. RESULTS: Computed tomography showed bulbous dilatation of the fundi of the internal auditory canals, incomplete bony separation between the basal turn of the cochleas and the lateral ends of the internal auditory canal, deficiency of the modiolus, enlarged first part of the facial nerve, and dilatation of the superior and the inferior vestibular nerve canal and the singular canal. Besides these characteristic findings, dilatation of the vestibular aqueduct was seen except in the man. Middle ear anomalies including oval and/or round window and/or stapes abnormalities were also detected in three affected patients. The carrier mothers had milder forms of some characteristic findings. CONCLUSIONS: Because of the risks of stapes surgery in X-linked deafness, recognition of the characteristic imaging features of these disorders is important. Especially in young patients with mixed hearing loss, temporal bone computed tomography should be performed before stapes surgery to avoid the complication of stapes gusher. Middle ear anomalies might be highly associated with X-linked deafness.