Mittelohrfehlbildungen sind eine heterogene Gruppe von angeborenen Anomalien mit unterschiedlichen
Ätiologien, Ausprägungen und klinischen Auswirkungen. Ein gründliches Verständnis
der genetischen und embryologischen Grundlagen sowie der chirurgischen Behandlungsstrategien
ist für ein optimales Ergebnis entscheidend. In diesem Beitrag werden auch Indikationen
sowie Vor- und Nachteile einer Hörimplantatversorgung bei solchen Fehlbildungen erläutert.
Abstract
Middle ear malformations (MEMs) represent a diverse group of congenital anomalies
with significant implications for auditory function. These malformations, which occur
in approximately 0.5 to 3% of conductive hearing loss cases, can arise from various
genetic and environmental factors. They often manifest unilaterally and may occur
in isolation or as part of a syndromic condition. MEMs are closely associated with
abnormalities of the external ear and less frequently with inner ear anomalies.
Embryologically, the middle ear develops from the first and second pharyngeal arches,
with interactions between the ectoderm and endoderm contributing to the formation
of essential structures such as the tympanic membrane, ossicles and Eustachian tube.
Disruptions in these developmental processes can lead to a spectrum of MEMs, ranging
from minor defects to severe malformations affecting multiple middle ear components.
Clinical management of MEMs requires a multidisciplinary approach, involving otolaryngologists,
pediatricians, and audiologists. Early intervention with appropriate hearing aids,
including conventional hearing aids and bone conduction devices, is essential to mitigate
the impact of conductive hearing loss on speech and language development, particularly
in children.
Surgical planning involves comprehensive preoperative assessment, including high-resolution
computed tomography imaging to evaluate middle ear anatomy, the facial nerve course,
and vascular anomalies. Traditional surgical approaches such as stapesplasty and tympanoplasty
remain mainstays for correcting specific middle ear defects, while advances in technology
have expanded the role of active middle ear implants in treating special cases.
In conclusion, MEMs represent a heterogeneous group of congenital anomalies with diverse
etiologies and clinical implications. A thorough understanding of their embryological
basis, genetic underpinnings, and surgical management strategies is crucial for optimizing
outcomes in affected individuals.
Schlüsselwörter
Audiologische Rehabilitation - Schallleitungsschwerhörigkeit - Gehörknöchelprothesen
- Hörimplantate - aktive Mittelohrimplantate - AMEI
Keywords
Audiologic Rehabilitation - Conductive Hearing Loss - Ossicle-Prostheses - Hearing
Implants - Active Middle Ear Implants - AMEI
