gms | German Medical Science

Congenital hearing disorders can be associated with aplasia or hypoplasia of the vestibulocochlear nerve [Ref. 1]. With monaural or binaural hearing disorders in children further paediatric and neurological diagnostics as well as radiological investigations are necessary apart from the paedaudiological diagnostics and treatment [Ref. 2]. In addition magnet resonance tomography (MRT) and cranial computer tomography are needed in high-grade hearing disorders as preliminary investigation when e.g. Cochlea implantation is planned. The necessity for these investigations sometimes is doubted because of expenditure of time, stationary stay and sedation especially when children are disabled or have severe comorbidities. Regarding the development and paedaudiological treatment of a young boy (unilateral, sinistral deafness, see figure 1 [Fig. 1]) we want to point out that a hearing loss has to be controlled by cranial tomography. The MRT accomplished in the Department for Neuroradiology at the University Hospital Aachen (Gyroscan Intera T15) resulted in aplasia and / or hypoplasia of the sinistral nervus vestibulocochlearis. Parts of the sinister facial nerve are still recognizable in the inner acoustic meatus (see figure 2 [Fig. 2]). The meaning of tomography for adequate paedaudiologic treatment especially in congenital hearing disorders [Ref. 3] is an important component of the therapy that has to be discussed with patients or adult relatives. Furthermore, technical increasingly optimized imaging results - morphologically seen - in improved diagnostic possibilities. With missing reproduction of Jewett-V-potentials in BERA-diagnostics and regarding hypo- or aplasia of the vestibulocochlear nerve in tomography - as a consequence - supply of hearing aids (including e.g. bone anchored hearing aids) with exception of the possibility of a CROS hearing aid supply is inadvisable. With contra lateral healthy and normal sense of hearing however the supply of a CROS hearing aid - particularly with children - is not always possible, since an acceptance often can not be attained by the patient and his relatives.