Rehabilitation for unilateral deafness - Narrative review comparing a novel bone conduction solution with existing options.

Patients with single sided deafness (SSD) struggle with sound localization and speech in noise. Existing treatment options include contralateral routing of signal (CROS) systems, percutaneous bone conduction hearing devices (BCHDs), passive transcutaneous BCHDs, active BCHDs, and cochlear implants. Implanted devices provide benefits in speech in noise compared to CROS devices. Percutaneous BCHDs transmit sound efficiently but have aesthetic drawbacks and skin complications. Scalp attenuation impacts passive transcutaneous BCHD performance. Active BCHDs overcome these issues and provide benefits for speech in noise. Cochlear implantation is the only existing option that restores binaural input but introduces electrical rather than acoustic stimuli to the deaf ear. Active BCHDs have been designed to maintain efficient sound transmission and avoid chronic skin irritation and cosmetic concerns that may occur with percutaneous BCHDs. Cochlear implantation may be a superior option for recently deafened SSD patients, though this requires further study. The duration of deafness, patient age and comorbidities, and a shared decision-making model among patients, surgeons, and audiologists should be considered in device selection. The aim of this manuscript is to review available devices, discuss surgical considerations for implantable devices, review available published results for speech in noise and sound quality with each device, and provide an overview to guide shared decision making for patients and providers. This review consolidates available literature and reviews experience with a newer active transcutaneous active BCHD available for use in the SSD population.

Cochlear implantation with ipsilateral petroclival chondrosarcoma.

OBJECTIVE: To highlight a case of cochlear implantation in the setting of ipsilateral petrous apex chondrosarcoma. BACKGROUND: A patient with bilateral progressive hearing loss was incidentally found to have a destructive right petrous apex lesion on computed tomography before cochlear implantation. The patient had no associated symptoms and a magnetic resonance imaging scan was obtained, narrowing the differential diagnosis. A middle cranial fossa approach was performed for synchronous biopsy of the lesion and cochlear implantation. RESULTS: Frozen sections revealed a low-grade chondroid lesion, and a Med-El Combi 40+ cochlear implant with a split electrode array was inserted via the middle fossa. Final pathologic examination revealed a Grade I chondrosarcoma. The patient suffered no complications postoperatively and was followed-up over 5 years with serial computed tomographic scans and clinical examinations. No additional treatment was administered. Eighteen months postoperatively, the patient experienced episodic vertigo. There were no new findings on computed tomography, and the vertigo improved with a low-salt diet. Otherwise, the patient had excellent hearing results, and the lesion has not progressed under observation. CONCLUSION: The implications of observing low-grade chondrosarcomas in well-selected patients and the unique aspect of cochlear implantation on the affected side are discussed.

Identification and characterization of choline transporter-like protein 2, an inner ear glycoprotein of 68 and 72 kDa that is the target of antibody-induced hearing loss.

The Kresge Hearing Research Institute-3 (KHRI-3) antibody binds to a guinea pig inner ear supporting cell antigen (IESCA) and causes hearing loss. To gain insight into the mechanism of antibody-induced hearing loss, we used antibody immunoaffinity purification to isolate the IESCA, which was then sequenced by mass spectroscopy, revealing 10 guinea pig peptides identical to sequences in human choline transporter-like protein 2 (CTL2). Full-length CTL2 cDNA sequenced from guinea pig inner ear has 85.9% identity with the human cDNA. Consistent with its expression on the surface of supporting cells in the inner ear, CTL2 contains 10 predicted membrane-spanning regions with multiple N-glycosylation sites. The 68 and 72 kDa molecular forms of inner ear CTL2 are distinguished by sialic acid modification of the carbohydrate. The KHRI-3 antibody binds to an N-linked carbohydrate on CTL2 and presumably damages the organ of Corti by blocking the transporter function of this molecule. CTL2 mRNA and protein are abundantly expressed in human inner ear. Sera from patients with autoimmune hearing loss bind to guinea pig inner ear with the same pattern as CTL2 antibodies. Thus, CTL2 is a possible target of autoimmune hearing loss in humans.