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

[dmvascon at usp.br]

Dear Prof. Walther

Autoinflammatory syndromes are frequently associated to neurological  
manifestations.
I remember of NALP3 (cryopyrin) associated diseases, which are  
frequently associated to sensorineural deafness, at least in its more  
severe forms (NOMID/CINCA - not the case in this particular patient)  
and Muckle-Wells syndrome (that commonly present deafness, periodic  
fever, skin manifestations and sometimes arthritis). Familiar cold  
urticaria is seldom associated with neurological manifestations.

Kind regards,

Dewton

Citando "Walther, Joachim-Ulrich  Prof.Dr." <Ju.Walther at med.uni-muenchen.de>:


> 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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