Surgical and Technical Modalities for Hearing Restoration in Ear Malformations

Abstract

Malformations of the external and middle ear often go along with an aesthetic and functional handicap. Independent of additional aesthetic procedures, a successful functional hearing restoration leads to a tremendous gain in quality of life for affected patients. The introduction of implantable hearing systems (bone conduction and middle ear devices) offers new therapeutic options in this field. We focus on functional rehabilitation of patients with malformations, either by surgical reconstruction or the use of different implantable hearing devices, depending on the disease itself and the severity of malformation as well as hearing impairment. Patients with an open ear canal and minor malformations are good candidates for surgical hearing restoration of middle ear structures with passive titanium or autologous implants. In cases with complete fibrous or bony atresia of the ear canal, the most promising functional outcome and gain in quality of life can be expected with an active middle ear implant or a bone conduction device combined with a surgical aesthetic rehabilitation in a single or multi-step procedure. Although the surgical procedure for bone conduction devices is straightforward and safe, more sophisticated operations for active middle ear implants (e.g., Vibrant Soundbridge, MED-EL, Innsbruck, Austria) provide an improved speech discrimination in noise and the ability of sound localization compared with bone conduction devices where the stimulation reaches both cochleae.

A Cross-Sectional Analysis of American Insurance Coverage of Prominauris Otoplasty

“…The first is in hearing loss, where an otoplasty performed in conjunction with an implantable hearing device can provide functional benefit to the patient's hearing. 21 This is often discussed in patients where the anatomical deformity of the ear contributes to the hearing loss itself, such as microtia and anotia. Though the physical features of prominauris does not normally cause hearing loss, it may be a marker of a global congenital syndrome, where hearing loss is an associated symptom.…”

“…For considering functional repair, an otoplasty can be utilized in ear deformities to improve a multitude of defects. The first is in hearing loss, where an otoplasty performed in conjunction with an implantable hearing device can provide functional benefit to the patient's hearing 21 . This is often discussed in patients where the anatomical deformity of the ear contributes to the hearing loss itself, such as microtia and anotia.…”

Implantierbare Knochenleitungs- und aktive Mittelohr-Hörsysteme

“…Auch heute noch werden einzelne Patienten, die aufgrund schwerwiegender Allgemeinerkrankungen nicht anderweitig rehabilitiert werden können, mit modernen Hörbrillen versorgt. In der Pädaudiologie hat die Versorgung mit einem Softband-KLHS vor allem in den ersten Lebensjahren immer noch einen hohen Stellenwert, beispielsweise zur zeitlichen Überbrückung, bis eine chirurgische Therapie durchgeführt oder ein implantierbares Mittelohrhörsystem bei Ohrfehlbildungen eingesetzt werden kann [9]. Bei einseitigen Schwerhörigkeiten ist die Akzeptanz einer solchen Versorgung dieser Altersgruppe jedoch in vielen Fällen eingeschränkt.…”