[PAGID] progressive loss of hearing in 6 year old boy

[Walther, Joachim-Ulrich Prof.Dr.]

To whom it may concern
 
Dear colleagues,
 
in the hope to direct my inquiry properly and adequately I put the following question to clinicians and immunologists involved in acquired deafness. 
I shall be extremely grateful for your comments and advice
 
Case:
Six year old boy, otherwise apart from mild gross motor retardation healthy.
Left ear found to be nearly completely deaf at five years, apparently after an underestimated loss of function over the years. Neonatal hearing test normal.
Now quickly developing (within a few months) loss of hearing contralaterally (low frequency spectrum). One course of steroid and osmotoc iv therapy given. During the few weeks since no improvement and no progression.    No family history, no consanguinity.
Clinically unremarkable.
No signs of inflammation and autoimmune activation in laboratory results.
Ophthalmological findings normal.
Audiologist´s interpretation:    autoimmune mediated loss of hearing. Advice: immunosuppressive therapy.
Our question: is this indicated ?
Genetic diagnosis is being considered, particularly to be able to avoid unnnecessary immunosuppression.
 
Many thanks for your help
 
  
Prof.Dr.med.J.-U.Walther M.Sc.
Allgemeinpädiatrischen Ambulanz 
der Kinderklinik und Kinderpoliklinik
im Dr.v.Haunerschen Kinderspital
der Ludwig-Maximilians-Universität
Lindwurmstraße 4
80337  München
 
Tel.:    089 / 5160-2811
Fax:      "        "   -7722
mail:    Ju.Walther at med.uni-muenchen.de <blocked::mailto:Ju.Walther at med.uni-muenchen.de> 
 
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