Susac Syndrome (SS) is a rare unique autoimmune underdiagnosed disorder characterized by the clinical triad of central nervous system (CNS) dysfunction, sensorineural hearing impairment, and branch retinal artery occlusion (BRAO). Objectives The purpose of reporting the case is to succinctly highlight this enigmatic condition with focus on otological and audiological aspect of SS and also to augment awareness of this disease among otolaryngologists neurologists, psychiatrists and ophthalmologists. Additionally we reviewed the otological menifestations and audiological findings related to SS. Methods A case of 38 year old female is presented with bilateral SNHL as the initial symptom of SS. The review of otological and audiological findings of previous 10 year case reports was executed using the PRISMA protocol on 20 cases. Results It was seen that inspite of the ear being one of the main organs involved in the SS very few had undergone detailed audiological assessment. Our review of literature showed that only 40% cases had done audiological assessment other than PTA, out of which only 5% had undergone complete audiological workup. 30% of the patients were having auditory symptoms as presenting chief complaint. Conclusion Otolyryngologists should keep SS in differentials when a case of SNHL of unknown etiology is encountered.We emphasize the use newer noninvasive Powered by Editorial Manager® and ProduXion Manager® from Aries Systems Corporation diagnostic tools like Optical coherence tomography (OCT), MRI and detailed audiological tests for timely diagnosis of SS. Further multidisciplinary assessment protocols should be used to prevent the development of severe sequelae.

Background: Tinnitus is a serious health condition. It can be debilitating and as such negatively affects a patient\'s quality of life. People with tinnitus often experience distress, depression, anxiety, frequent mood swings, sleep disturbances, irritability, frustration, poor concentration, and possible suicidal thoughts or actions. Objective: The goal of this article is to introduce an acupuncture system, based on neurophysiology and termed Neuropuncture, as a possible effective treatment method for tinnitus accompanied with and/or secondary to hearing loss. The treatment protocol works by targeting the greater auricular nerve, trigeminal nerve, cervical plexus, and auditory cortex to neuromodulate, neurorehabilitate, and neuroregulate the nervous system and repair the nerve damage. Design: Three case studies are presented herein as examples. They are based on neurophysiologic mechanism of tinnitus and hearing loss, treatment principle, treatment methods, and subjective and clinically objective tests. Electroacupuncture protocols used various frequencies with microcurrent and millicurrent stimulation. Conclusions: Neuropuncture system is an effective treatment for patients with acute and chronic subjective tinnitus and hearing loss. Results showed reduction of tinnitus and partial restoration of hearing loss. Further research and possible large-scale trial studies are suggested.

BACKGROUND: To date, cCMV represents the most frequent non-genetic congenital cause of permanent sensorineural hearing loss (SNHL) in childhood and the leading infectious cause of developmental and neurologic disabilities. The aim of this paper is to describe the outcome of cochlear implantation in children affected by severe-to-profound sensorineural hearing loss, due to a symptomatic or asymptomatic cCMV infection, particularly comparing their performance results to that of matched mutated Connexin 26 (Cx26) implanted patients.
METHODS: Retrospective case control study. The clinical data of symptomatic cCMV and asymptomatic cCMV patients were collected and compared to those of Cx26 patients matched for age and pre-CI (cochlear implant) linguistic category; all subjects were affected by bilateral severe-to-profound SNHL and were treated by CI and speech therapy rehabilitation. The Speech Perception Category, the language stage and the linguistic level scores, at 6 months, 1 year, and 3-4 years after CI of the three groups (symptomatic cCMV, asymptomatic cCMV and Cx26 mutation) were collected and compared.
RESULTS: Statistical analysis did not show any significant difference in pre-CI perception category and linguistic level among the three groups; the symptomatic cCMV group showed a statistically worse performance of the language stage over time (p = 0.017).
CONCLUSIONS: Our data support that children affected by cCMV have improved language abilities over time, although the symptomatic cCMV group achieved a lower language stage 3-4 years after CI compared to the asymptomatic cCMV and Cx26 mutation groups. Nonetheless, to date, CI supported by speech therapy can be considered an effective intervention for children affected by cCMV-related severe-to-profound hearing loss.

We present a case of an otherwise healthy 20-month-old with congenital sensorineural hearing loss. CT and MR imaging demonstrated bilateral asymmetrically severe hypoplasia of the internal auditory canals and vestibulocochlear nerves. Additional developmental inner ear anomalies were present in this patient, including unilateral semicircular canal hypoplasia and suspected bilateral cochlear hypoplasia. The patient retained normal facial nerve function bilaterally. We highlight the current research and understanding of congenital IAC abnormalities.

BACKGROUND: Sudden sensorineural hearing loss (SNHL) after nonotologic surgery is unusual, with most occurrences reported in patients having cardiopulmonary bypass. Reports of SNHL after nonotologic noncardiac surgery are rare, particularly after spine surgery. In patients undergoing subarachnoid anesthesia or intradural surgery, loss of cerebrospinal fluid pressure can result in hearing loss and cranial nerve palsy.
METHODS: A 70-year-old male patient had ankylosing spondylitis with an Anderson lesion T11-12 with diabetes mellitus, hypertension, and American Spinal Injury Association Impairment Scale-A neurology. He underwent posterior stabilization of T10-L1 and developed a profound, painless, left-sided hearing deficit shortly afterward. An otorhinolaryngology consultation confirmed SNHL. An 82-year-old male reported claudication with L4-5 LCS and type 2 diabetes mellitus. Post L4-5 transforaminal lumbar interbody fusion, he complained of hearing loss in his left ear, confirmed by an otolaryngologist. A 72-year-old diabetic male had tuberculous spondylodiskitis of D2-3 with an epidural abscess. An emergent decompression and stabilization C7-D5 bone grafting at D2-3 was done. In the early postoperative period, the patient complained of marked hearing impairment on the right side. Audiometry indicated SNHL in his right ear.
RESULTS: A course of intravenous corticosteroids was instituted in the first and second patients for severe hearing loss. Steroids were not given to the third patient in view of his Pott spine. No improvement in hearing impairment occurred until the latest follow-up in all patients.
CONCLUSIONS: Sudden-onset hearing loss post spine surgery is disastrous with multiple etiologies and remains poorly understood. Timely evaluation by an otolaryngologist and audiologist is recommended. The role of corticosteroids in treating SNHL is still unclear.

This retrospective, observer blinded case-control study aims to compare the prevalence of neurovascular conflicts (NVCs) of the vestibulocochlear nerve and the anterior inferior cerebellar artery (AICA) in patients presenting with clinical signs of acute vestibular neuritis with and without subsequent objective vestibular function loss (VFL). 58 acute cases of clinically suspected acute vestibular neuritis were investigated with same day cranial MRI at a tertiary referral centre and compared to 61 asymptomatic controls. The prevalence of NVCs in cases with objective VFL were also compared to cases without VFL. Radiologists described the NVC as \"no contact\" (Grade 0), \"contact < 2 mm\" (Grade 1), \"contact > 2 mm\" (Grade 2) and \"vascular loop presence\" (Grade 3) without knowledge of neurotological data. Neurotological data was collected without knowledge of MRI findings. Vestibular function was tested by bithermic caloric irrigation. 26 cases (45%) showed caloric VFL (Group A), whereas 32 (55%) exhibited no VFL (Group B). Group A included 13 cases with NVCs (50%), Group B included 26 NVC cases (82%) (p = 0.012) and the control group included 16 individuals (26%) (p < 0.001 for comparison of all 3 groups). Group B had a significantly higher NVC-Grading than Group A (p = 0.009). There was no statistically significant association between NVCs and either SNHL or tinnitus (p > 0.05). Our results suggest that patients presenting with clinical signs of acute vestibular neuritis who show symmetrical caloric vestibular function test results have a significantly higher NVC prevalence in the cerebellopontine angle.
Il presente studio retrospettivo a singolo cieco si pone come obbiettivo quello di valutare in che percentuale di casi di pazienti che si presentano con sintomatologia compatibile con neurite vestibolare acuta, con e senza perdita oggettiva della funzione vestibulare (VFL), sia presente un conflitto neurovascolare fra il nervo vestibolococleare e la arteria cerebellare anteroinferiore (AICA). 58 pazienti con sintomatologia suggestiva per neurite vestibolare acuta, valutati con RMN presso un centro di terzo livello, sono stati confrontati con 61 pazienti asintomatici. I radiologi hanno dato valutato la presenza di conflitto neurovascolare, in assenza di dati clinici, conferendo ai rilievi oggettivi una valutazione in una scala da 0 a 3 a seconda che il contatto fosse: nesuno; inferiore a 2 mm; superiore ai 2 mm; presenza di vacular loop. I reperti neurootologici sono stati quindi raccolti all’oscuro del risultato dell’imaging. La funzione vestibolare è stata testata con prova calorica bitermica. Alla prova calorica 26 casi (45%) hanno mostrato segni oggettivi di deficit vestibolare (Gruppo A), 32 casi (55%) non hanno invece mostrato alcun deficit labirintico (Gruppo B). Il gruppo A ha incluso 13 casi (50%) con evidenza di conflitto neurovascolare (NVC), il gruppo B ha incluso 26 casi con NVC (82%) (p = 0.012) mentre i controlli hanno incluso 16 casi con NVC (26%). La differenza fra i tre gruppi ha mostrato significatività statistica (p<0.001). Il Gruppo B ha mostrati un associazione con un grading di conflitto piu elevato rispetto al Gruppo A (p = 0.009). La presenza di NVC non ha avuto un associazione statisticamente significativa ne con la presenza di SNHL ne con la presenza di acufene (p > 0.05). I nostri dati indicano che la presenza di conflitti neurovascolari a livello dell’angolo pontocerebellare è superiore in quei pazienti che in presenza di una sintomatologia compatibile con neurite vestibolare acuta abbiano una funzionalità simmetrica alla prova calorica.