Migraine and Cochlear Symptoms.

Migraine is one of the most common and highest burdens of disease. As a primary cerebral dysfunction illness, migraine might exhibit other system-related symptoms, including vestibular and cochlear symptoms. With the publication of the diagnostic criteria of vestibular migraine, the link between migraine and vestibular symptoms became clear. However, the relationship between migraine and cochlear symptoms is far from straightforward. Therefore, we focus on the correlation between migraine and deafness, sudden sensorineural hearing loss, acute tinnitus, and chronic tinnitus to better understand the relationship between migraine and cochlear symptoms.

[Cochleo-vestibular lesions and prognosis in patients with profound sudden sensorineural hearing loss: a comparative analysis].

Objective: To investigate the characteristics of cochleo-vestibular dysfunction in patients with profound sudden deafness, and the prognosis of inner ear hemorrhage. Methods: From January 2017 to December 2018, 92 inpatients with profound sudden sensorineural hearing loss were enrolled in the Department of Otorhinolaryngology, First Affiliated Hospital of Sun Yat-sen University. Our studied patients included 47 males and 45 females, aged 20-78 (39.3±6.1) years. According to the results of inner ear magnetic resonance imaging (MRI), the patients were divided into two groups: inner ear hemorrhage group and non-inner ear hemorrhage group. The clinical features, vestibular tests and audiological examination results during follow up were compared between the two groups. SPSS 22.0 software was used for statistical analysis. Results: The inner ear hemorrhage group consisted of 32 cases (34.8%, 32/92), all of whom complained of vertigo (100%, 32/32). Simultaneous vertigo attack and hearing loss occurred in 78.1% of this group (24/32). Neither semicircular canals function, nor cervical vestibular evoked myogenic potential (c-VEMP), nor ocular vestibular evoked myogenic potential (o-VEMP) in the affected side was normal (100%, 32/32). The rates of benign paroxysmal positional vertigo (BPPV) and disequilibrium were 37.5% (12/32) and 25.0% (8/32) respectively. Hearing improved in 28.1% (9/32) two weeks after treatment, and became stable at one month's follow up. In 60 cases without inner ear hemorrhage, 58.3% of them (35/60) experienced vertigo, which occurred simultaneously with hearing loss in 21 patients (60%, 21/35). The abnormal rates of semicircular canals function, c-VEMP and o-VEMP were 71.6% (43/60), 78.3% (47/60) and 66.7% (40/60), respectively. The incidence of BPPV was 16.7% (10/60) and 8.3% (5/60) in cases with disequilibrium. Hearing improved in 58.3% (35/60) two week after treatment, and became stable at three months' follow up. Significant difference was found in either vertigo rate, or simultaneous vertigo/hearing loss rate, or abnormal c-VEMP/o-VEMP rates, or accompanying BPPV, or disequilibrium rates between the two groups (P<0.05 each). Moreover, we observed better hearing recovery in non-inner ear hemorrhage group in the two weeks, one month, three months and six months' follow up, when compared with those in inner ear hemorrhage groups (P<0.05 each). Conclusions: Inner ear hemorrhage is associated with more severe cochlea-vestibular lesion and poorer prognosis, in comparison to the non-inner ear hemorrhage,in patients with profound sudden sensorineural hearing loss.

Remission of Recurrent Cochlear Hydrops Associated With Bromocriptine Treatment for Macroprolactinoma.

Objective Current recommendations for cochlear hydrops treatment include systemic glucocorticoids and diuretics. Cochlear cells express dopamine receptors, although their role is unknown in the pathophysiology of cochlear hydrops. Case Description We report the case of remission of recurrent right-sided cochlear hydrops in a young male patient treated with bromocriptine due to pituitary macroprolactinoma. Transient improvement was observed after oral steroid and diuretic treatment, but cochlear hydrops recurred until the dose of bromocriptine was increased to 10 mg daily. Conclusion Bromocriptine may stimulate dopamine receptors in cochlear cells with potential therapeutic role in patients with cochlear hydrops. There are no widely accepted and effective treatments for endolymphatic hydrops, and identifying potential new and efficacious therapeutics is of high relevance.

Carotid-cochlear dehiscence: a dangerous mimicker of inner ear pathologies.

A 67-year-old woman was referred to the otolaryngology service after presenting to the emergency department for dizziness and loss of balance. She reported several similar episodes over the past years. Physical examination was unremarkable. A temporal bone CT scan revealed dehiscence between the bony carotid canal and the cochlea resulting in the diagnosis of carotid-cochlear dehiscence (CCD). CCD is an extremely rare condition involving the thinning of the bony canal separating the internal carotid artery from the cochlea. CCD is best diagnosed with temporal bone CT scan. Treatment options include observation as well as chemical or surgical labyrenthectomy. Despite similar clinical and diagnostic characteristics of reported CCD cases, general trends and consensus on treatment options cannot be ascertained due to the extreme rarity of this condition. Regardless of these limitations, CCD is a critical diagnosis as it mimics other inner ear conditions and poses a potential, significant surgical risk for the otolaryngologist.

Inner ear and retrocochlear pathology on magnetic resonance imaging for sudden and progressive asymmetrical sensorineural hearing loss.

BACKGROUND: In sudden and asymmetrical progressive sensorineural hearing loss (SNHL), magnetic resonance imaging (MRI) is required to evaluate retrocochlear pathology and, with recent advances in MRI techniques, inner ear pathology. Given the limited literature regarding inner ear pathology associated with SNHL, we aimed to assess the incidence of retrocochlear and inner ear pathology, and congenital malformation on MRI in sudden SNHL (SSNHL) and progressive SNHL. METHODS: A total of 987 acoustic neuroma (AN) protocol MRI internal acoustic meatus studies performed at our institution to investigate SNHL between January 2013 and December 2015 inclusive were identified. Following categorization for indication of SSNHL versus progressive asymmetrical SNHL, MRIs with retrocochlear or inner ear abnormality, congenital malformation or other otology-related abnormality were identified, and further data were collected for these patients including patient demographics, associated symptomatology, management and outcomes. RESULTS: In SSNHL, aetiological abnormality on MRI was identified for 6.9% patients with AN present on 4% overall. 3.2% of MRIs for progressive asymmetrical SNHL identified a causative lesion with 2.3% of scans overall diagnosing AN. The incidence of congenital inner ear malformation on MRI in the setting of SSNHL and progressive asymmetrical SNHL are 1.7% and 0.6%, respectively. CONCLUSION: This is the first retrospective study of inner ear MRI abnormalities in both SSNHL and progressive asymmetrical SNHL in Australia and one of the largest cohorts published in the literature to date. MRI must be performed in the setting of SNHL to ensure aforementioned and rarer causative lesions are identified.

Vestibular Dysfunction in Patients With Superficial Siderosis of the Central Nervous System.

OBJECTIVE: To describe the vestibular function in patients with superficial siderosis of the central nervous system (SSCN). STUDY DESIGN: Retrospective analysis. SETTING: Tertiary referral center. PATIENTS: Ten consecutive patients with SSCN. This study is the largest case series of SSCN in which detailed neuro-otological findings, including electronystagmography recording, video head impulse test (vHIT), and posturography, were described. INTERVENTIONS: Audiological and neuro-otological examinations, including pure-tone audiometry, distortion product otoacoustic emissions, speech audiometry, auditory brainstem responses, electronystagmography recording, vHIT, and posturography. MAIN OUTCOME MEASURES: Pure-tone average, DP level, maximum speech discrimination score, interpeak latency between auditory brainstem responses waves I and V, eye tracking test, examination of optokinetic nystagmus, caloric response, visual suppression, vestibulo-ocular reflex gains, total center of pressure path length, and Romberg's ratio. RESULTS: Audiological examinations suggested that the sensorineural hearing loss was of a cochlear etiology in 3 ears, a retrocochlear etiology in 11 ears, and a combined cochlear and retrocochlear etiology in 6 ears. Neuro-otological examinations revealed that eight out of nine patients had cerebellar disorders, while all patients also had peripheral vestibular dysfunction. CONCLUSION: In addition to cerebellar disorders, SSCN patients suffer from severe peripheral vestibular dysfunction, which can exacerbate the patient's imbalance. When otolaryngologists encounter patients with distinctly progressive sensorineural hearing loss and imbalance, they should include SSCN in the differential diagnosis and perform neuro-otological examinations, including an electronystagmography recording and vHIT and brain magnetic resonance imaging.

Preclinical and clinical otoprotective applications of cell-penetrating peptide D-JNKI-1 (AM-111).

There is a growing interest in the auditory community to develop novel prophylactic and therapeutic drugs to prevent permanent sensorineural hearing loss following acute cochlear injury. The jun-N-terminal protein kinase (JNK) pathway plays a crucial role in acute sensory hearing loss. Blocking the JNK pathway using the cell-penetrating peptide D-JNKI-1 (AM-111/brimapitide) has shown promise as both a prophylactic and therapeutic agent for acute cochlear injury. A number of pre-clinical and clinical studies have determined the impact of D-JNKI-1 on acute sensorineural hearing loss. Given the inner-ear selective therapeutic profile, local route of administration, and ability to diffuse across cellular membranes rapidly using both active and passive transport makes D-JNK-1 a promising oto-protective drug. In this review article, we discuss the application of D-JNKI-1 in various auditory disorders as well as its pharmacological properties and distribution in the cochlea.

Severe obstructive sleep apnea is associated with cochlear function impairment.

PURPOSE: The purpose of this study is to investigate the association between obstructive sleep apnea (OSA) with middle ear acoustic transference and cochlear function. METHODS: Male individuals with and without mild, moderate, and severe OSA according to standard criteria of full polysomnography and no co-morbidities were studied. Subjects with BMI ≥40 kg/m2, present or past treatment for OSA, with heart failure, diabetes, hypertension, dyslipidemia, stroke, use of chronic medications, and previous history of risk for hearing loss were excluded. All subjects were submitted to full polysomnography, evaluation of wideband acoustic immittance by energy of absorbance (EA), and distortion product otoacoustic emissions (DPOAE). RESULTS: We studied 38 subjects (age 35.8 ± 7.2 years, BMI 28.8 ± 3.8 kg/m2) divided into no OSA (n = 10, age 33.6 ± 6.4 years, BMI 26.9 ± 4.1 kg/m2), mild (n = 11, age 32.8 ± 2.9 years, BMI 28.5 ± 3.5 kg/m2), moderate (n = 8, age 34.1 ± 6.8 years, BMI 29.6 ± 3.3 kg/m2), and severe OSA (n = 9, age 41.2 ± 9.2 years, BMI 30.5 ± 3.8 kg/m2). EA was similar between groups. In contrast, patients with severe OSA presented significantly lower DPOAE amplitudes when compared to the control, mild, and moderate OSA groups (p ≤ 0.03, for all comparisons). CONCLUSIONS: Acoustic transference function of middle ear is similar in adults with and without OSA. Severe OSA is independently associated with cochlear function impairment in patients with no significant co-morbidities.

Autologous intratympanic blood patch for presumed perilymphatic fistulas.

OBJECTIVE: To assess an alternative to bed rest and surgery for suspected perilymphatic fistulas using intratympanic blood injections. METHOD: A review was conducted of patients' history, physical and audiometric data, before and after treatment by intratympanic blood injections performed from 2009 to 2015. RESULTS: Twelve ears were identified, with trauma associated with air travel, water sports or nose blowing. Ten of these cases had hearing loss, six had vestibular symptoms. Four cases had audiological and vestibular symptoms, two had vestibular symptoms only, and six had audiological symptoms only. Time-to-treat varied from 1 day to 30 days. Magnetic resonance imaging scans were obtained for five cases. Ten cases received steroids. Six out of seven cases showed improvement of hearing loss. Five cases showed positive fistula test results, four with documented resolution. Seven cases had full resolution of all symptoms, four had near-full resolution and one had no improvement. CONCLUSION: Intratympanic blood injections offer an effective alternative to conservative or surgical therapy. Advantages include sooner time-to-treat, lower financial costs and decreased psychosocial burdens. It allows a more flexible and liberal use of a potential definite treatment for perilymphatic fistula.

Electrococleografía extratimpánica en una población normal. Estudio descriptivo / Extra-tympanic electrocochleography in a normal population. A descriptive study

Introducción y objetivos: La electrococleografía extratimpánica es un registro elecrofisiológico que refleja la actividad eléctrica acontecida en la cóclea tras un estímulo sonoro. Se obtiene mediante la aplicación del estímulo en el conducto auditivo externo y el registro de la actividad eléctrica mediante electrodos de superficie. Dada la escasa literatura existente acerca de la exploración electrococleográfica normal en nuestro medio, este estudio busca regularizar los valores obtenidos mediante esta exploración en sujetos sin enfermedad otoneurológica y explicar detalladamente el proceso mediante el cual se obtiene el registro. Métodos: Exploración mediante electrococleografía extratimpánica de 60 oídos sin enfermedad otoneurológica y análisis estadístico de los resultados obtenidos. De ellos, 30 oídos fueron explorados a 90dB mientras que otros 30 oídos fueron estimulados a 80dB. Resultados: Se muestran los valores medios de amplitud y latencia del potencial de sumación y del potencial de acción, así como las latencias medias de la onda I y la onda II. Asimismo, se presenta el cálculo del cociente potencial de sumación/potencial de acción. Obtenidos estos resultados se comparan en función de la intensidad del estímulo, del sexo del paciente, del oído estudiado y del grupo etario. Conclusiones: Este estudio recopila datos sobre la electrococleografía en una población normal. Los valores obtenidos están en el rango de los valores normales de otros países, expuestos en la literatura internacional. Estos datos pueden ser muy útiles como referencia a la hora de valorar exploraciones en pacientes con dolencias que afectan la estructura o la función coclear (AU)

Introduction and objectives: Extra-tympanic electrocochleography is an electrophysiological register obtained after stimulating the cochlea with an audible stimulus. This stimulus is applied using an earphone over the external auditory canal, while the electrical activity is registered by surface electrodes. There are few studies that analyse normal electrocochleography in our environment. Thus, the main objective of our study was to regularize the values obtained with electrocochleography in ears without any otoneurological diseases. We explain in detail the process of obtaining the register. Methods: Sixty healthy ears were studied by extratympanic electrocochleography. Statistical results were analysed. While 30 ears were studied with a stimulus at 90dB, another 30 ears were studied with a stimulus at 80dB. Results: Summating potential and action potential latencies and amplitudes were measured. Summating potential/action potential ratios were calculated. Wave I and wave II latencies were also determined. These results were analysed in function of stimulus intensity, patient gender, patient age group and ear side studied. Conclusions: This study collected extra-tympanic electrocochleography data in a normal population and the results were in the range of other international studies obtained in other countries. These data can be used as a reference to evaluate illnesses that affect cochlear structure or functions (AU)

Perilymph fistula: the patients' experience.

OBJECTIVE: This study aimed to assess the experiences and outcomes of patients who underwent surgical repair of a perilymph fistula in Norfolk, UK. METHODS: The study involved a retrospective questionnaire-based patient survey and case note review of patients who had undergone tympanotomy and perilymph fistula repair between 1998 and 2012 in two district general hospitals. RESULTS: Fourteen patients underwent 20 procedures, of whom 7 completed the pre- and post-operative Vertigo Symptom Scale. In five patients, there was no obvious precipitating cause. Perilymph fistula was precipitated by noise in one patient, by a pressure-increasing event in six patients and by trauma in two patients. The Vertigo Symptom Scale scores showed a statistically significant improvement following surgical repair, from a median of 67 (out of 175) pre-operatively to 19 post-operatively. CONCLUSION: In selected patients with vertigo, perilymph fistula should be considered; surgical repair can significantly improve symptoms.

The role of fissula ante fenestram in unilateral sudden hearing loss.

The cause of unilateral sudden sensorineural hearing loss (SNHL) remains unclear in many clinical cases. Perilymphatic leakage through a fissula ante fenestram (FAF) fistula is one possible reason. We present four clinical cases with proven FAF fistula, discovered during surgical exploration. All patients experienced partial hearing recovery after surgical coverage of the fistula. We suggest FAF as a possible site for perilymphatic leakage, representing an anatomical correlate for sudden unilateral SNHL. We recommend early exploratory tympanotomy with special attention to the bony region, anterior to the oval window, in cases of severe sudden SNHL and suspected FAF.

Endolymphatic hydrops in superior canal dehiscence and large vestibular aqueduct syndromes.

OBJECTIVES/HYPOTHESIS: Pathologic third window lesions, such as superior semicircular canal dehiscence syndrome (SCDS) or large vestibular aqueduct syndrome (LVAS), cause several auditory and vestibular symptoms, which might affect perilymphatic pressure and induce endolymphatic hydrops (EH). In this study, the existence of EH in subjects with SCDS or LVAS was investigated using contrast-enhanced magnetic resonance imaging (MRI). STUDY DESIGN: Case series at university hospital. METHODS: Seventeen ears from nine subjects who were diagnosed as having SCDS (five ears from three cases) or LVAS (12 ears from six cases) were studied. Ears were evaluated by 3-T MRI performed 4 hours after intravenous injection of gadodiamide hydrate. Imaging data concerning the degree of EH in the cochlea and the vestibule were compared with clinical symptoms and hearing levels for all ears. RESULTS: All ears showed air-bone gaps at low frequencies on pure tone audiometry. None of the subjects with SCDS had episodes of acute sensorineural hearing loss (SNHL) or vestibular symptoms, except for one patient who complained of head vibration induced by loud noise. Conversely, five of six subjects with LVAS had episodes of acute SNHL or vestibular symptoms. Four of five ears with SCDS showed severe EH in the cochlea, and two ears showed mild EH in the vestibule. All ears with LVAS showed mild to severe EH in both the cochlea and vestibule. CONCLUSIONS: The present study demonstrated the existence of EH in ears with pathologic third window lesions, which might affect patients' auditory or vestibular symptoms. LEVEL OF EVIDENCE: 4 Laryngoscope, 126:1446-1450, 2016.

Cochlear Nerve Stimulation in the Internal Auditory Canal in Ossified Cochlea: A Case Study.

OBJECTIVE: To present the first reported case of intraneural direct cochlear nerve stimulation in a human being. STUDY DESIGN: This is a case report. RESULTS: A 23-year-old patient with bilateral progressive hearing loss associated with bilateral complete semicircular canal aplasia and ossified cochleas underwent cochlear implantation. During surgery, a patent cochlear lumen could not be found, and the array was positioned in the internal auditory canal adjacent to the cochlear nerve. Against our expectations, an assiduous rehabilitation and frequent fitting adjustments have led to a word recognition score, in open set speech with lip reading, of 18/25 and acceptable frequency discrimination. CONCLUSIONS: We are aware that this was an anomalous use of the cochlear implant, and it is not our aim to suggest a new indication for cochlear array positioning. However, this case shows that auditory perception, to some degree, can be obtained with intraneural direct cochlear nerve stimulation.

Deafness in cochlear and auditory nerve disorders.

Sensorineural hearing loss is the most common type of hearing impairment worldwide. It arises as a consequence of damage to the cochlea or auditory nerve, and several structures are often affected simultaneously. There are many causes, including genetic mutations affecting the structures of the inner ear, and environmental insults such as noise, ototoxic substances, and hypoxia. The prevalence increases dramatically with age. Clinical diagnosis is most commonly accomplished by measuring detection thresholds and comparing these to normative values to determine the degree of hearing loss. In addition to causing insensitivity to weak sounds, sensorineural hearing loss has a number of adverse perceptual consequences, including loudness recruitment, poor perception of pitch and auditory space, and difficulty understanding speech, particularly in the presence of background noise. The condition is usually incurable; treatment focuses on restoring the audibility of sounds made inaudible by hearing loss using either hearing aids or cochlear implants.

Loss of auditory activity modifies the location of potassium channel KCNQ5 in auditory brainstem neurons.

KCNQ5/Kv7.5, a low-threshold noninactivating voltage-gated potassium channel, is preferentially targeted to excitatory endings of auditory neurons in the adult rat brainstem. Endbulds of Held from auditory nerve axons on the bushy cells of the ventral cochlear nucleus (VCN) and calyces of Held around the principal neurons in the medial nucleus of the trapezoid body (MNTB) are rich in KCNQ5 immunoreactivity. We have previously shown that this synaptic distribution occurs at about the time of hearing onset. The current study tests whether this localization in excitatory endings depends on the peripheral activity carried by the auditory nerve. Auditory nerve activity was abolished by cochlear removal or intracochlear injection of tetrodotoxin (TTX). Presence of KCNQ5 was analyzed by immunocytochemistry, Western blotting, and quantitative reverse transcription polymerase chain reaction. After cochlear removal, KCNQ5 immunoreactivity was virtually undetectable at its usual location in endbulbs and calyces of Held in the anteroventral CN and in the MNTB, respectively, although it was found in cell bodies in the VCN. The results were comparable after intracochlear TTX injection, which drastically reduced KCNQ5 immunostaining in MNTB calyces and increased immunolabeling in VCN cell bodies. Endbulbs of Held in the VCN also showed diminished KCNQ5 labeling after intracochlear TTX injection. These results show that peripheral activity from auditory nerve afferents is necessary to maintain the subcellular distribution of KCNQ5 in synaptic endings of the auditory brainstem. This may contribute to adaptations in the excitability and neurotransmitter release properties of these presynaptic endings under altered input conditions.

Perilymphatic fistula of the round window after whiplash injury: another cause of inner ear conductive hearing loss.

Perilymphatic fistula is usually associated with sudden or fluctuating sensorineural hearing loss. We present a case of whiplash injury in a patient who showed conductive hearing loss at low frequencies due to a perilymphatic fistula occurring in the round window. Although no middle ear pathology was found, the symptoms and laboratory findings were mimicking the so called "third mobile window" phenomenon, but without the presence of inner ear dehiscence. Following early surgical exploration with patching of the round window the hearing was restored and the patient was free of symptoms.