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Bilateral, mixed hearing loss with a predominant sensorineural component in Larsen's syndrome

Published online by Cambridge University Press:  18 February 2008

I O Ott  and
P R Issing
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Abstract

Background:

Larsen's syndrome is primarily known as an orthopaedic disease. However, it has been found to be associated with conductive hearing loss caused by ossicular malformation. The possibility of a sensorineural hearing impairment as an additional part of this syndrome has been rather neglected in the past.

Method:

Case report and literature review.

Patient and results:

We present a teenage boy suffering from typical Larsen's syndrome. Despite no history of recurrent middle-ear infections, he showed a bilaterally symmetrical, mixed hearing loss dominated by an extensive sensorineural component. In order to review the corresponding literature, a Medline search was performed using the criteria ‘Larsen's syndrome’ and ‘hearing loss/deficit’.

Conclusion:

The literature review showed the presented case to be the first sufficiently documented report describing a predominant sensorineural hearing loss in a Larsen's syndrome patient. Although a specific association of Larsen's syndrome with sensorineural hearing loss is still speculative, this case might give grounds for further examinations of this rare finding.

Keywords

Larsen's SyndromeHearing Loss, SensorineuralOssicular Malformation
Type
Research Article
Information
The Journal of Laryngology & Otology , Volume 122 , Issue 3 , March 2008 , e6
Copyright
Copyright © JLO (1984) Limited 2008

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References

1 Larsen, JJ, Schoffstaedt, ER, Bost, FC. Multiple congenital dislocations associated with characteristic facial abnormality. J Pediatr 1950;37:574CrossRefGoogle ScholarPubMed
2 Ronnigen, H, Bjerkrein, I. Larsen's syndrome. Acta Orthop Scand 1978;49:138Google Scholar
3 Habermann, ET, Sterling, A, Dennis, RI. Larsen's syndrome, a heritable disorder. J Bone Joint Surg 1976;58:558CrossRefGoogle ScholarPubMed
4 Salmon, MA, Lindenbaum, R. Harelip in Larsen's syndrome. Lancet 1973;i:318CrossRefGoogle Scholar
5 Tsang, MCK, Ling, JKH, Chow, SK. Oral and craniofacial morphology of a patient with Larsen syndrome. J Craniofac Gen Dev Biol 1986;6:357–62Google ScholarPubMed
6 Grundfast, KM, Mumtaz, A, Kanter, R, Pollack, M. Tracheomalacia in an infant with multiplex congenital (Larsen's) syndrome. Ann Otol 1981;90:303–6Google Scholar
7 Steel, HH, Kohl, EJ. Multiple congenital dislocations associated with other skeletal anomalies in three siblings. J Bone Joint Surg 1972;54:78CrossRefGoogle ScholarPubMed
8 Miz, GS, Engler, GL. Atlanto-axial subluxation in Larsen's syndrome. Spine 1987;12:411–12CrossRefGoogle ScholarPubMed
9 Swensson, R, Linnebur, AC, Paster, SB. Striking aortic root dilatation in a patient with the Larsen syndrome. J Pediatr 1975;86:914–15Google Scholar
10 Kiel, EA, Frias, JL, Victoricu, BE. Cardiovascular manifestations in the Larsen syndrome. Pediatrics 1983;71:942–6CrossRefGoogle ScholarPubMed
11 Ventruto, V, Festa, B, Sebastio, L, Sebastio, G. Larsen syndrome in two generations of an Italian family. J Med Genet 1976;13:538–9Google Scholar
12 Renault, F, Arthuis, M, Rethore, MO, Lafourcade, J. Larsen's syndrome [in French]. Arch Fr Pediatr 1982;39:35–8Google Scholar
13 Stanley, CS, Thelin, JW, Miles, JH. Mixed hearing loss in Larsen syndrome. Clin Genet 1988;33:395–8Google Scholar
14 Herrmann, HC, Kelly, JH, Fried, MP, Strome, M. The association of a hearing deficit with Larsen's syndrome. J Otolaryngol 1981;10:45–8Google ScholarPubMed
15 Horn, KL, Pai, V, Beauparlant, PA. Stapedotomy in Larsen's syndrome. Am J Otol 1990;11:205–6Google ScholarPubMed
16 Maack, RW, Muntz, HR. Ossicular abnormality in Larsen's syndrome: a case report. Am J Otolaryngol 1991;12:51–3CrossRefGoogle ScholarPubMed
17 Kaga, K, Suzuki, J, Kimizuka, M. Temporal bone pathology of two infants with Larsen's syndrome. Int J Pediatr Otorhinolaryngol 1991;22:257–67CrossRefGoogle ScholarPubMed
18 Percin, EF, Percin, S, Sezgin, I, Akbas, AK. Larsen's syndrome with mixed-type hearing loss. Acta Orthop Belg 1994;60:328–31Google Scholar