Audiologic results in patients with Moebiüs sequence

Griz, Silvana Maria Sobral; Cabral, Mariana Petry; Azevedo, Graziela de Souza; Ventura, Liana

doi:10.1016/j.ijporl.2007.06.002

Cited by 6 publications

(4 citation statements)

References 22 publications

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“…We did not find any evident muscular defects. Audiological testing findings were compatible with the results reported by Griz et al and Bezzera et al [27,28]. Low serum testosterone and increased LH and FSH were found and confirmed hypogonadotropic hypogonadism in our patient, which is rather uncommon in MBS.…”

Section: Mo or Rp Ph Ho Ol Lo Og Gi Ic Ca Al L P Ph He En No Ot Ty supporting

confidence: 92%

The natural history of Möbius syndrome in a 32-year-old man

Chorąży

Leśniewicz

Posmyk³

et al. 2011

Neurologia i Neurochirurgia Polska

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Möbius syndrome (OMIM#157900) is an extremely rare congenital entity involving bilateral or unilateral palsy of the facial nerve, usually with dysfunction of other cranial nerves (second, third, fifth, sixth, ninth, tenth and twelfth). It was estimated that Möbius syndrome occurs in 1 of 50 000 live births. The aetiology and the pathogenesis of the syndrome remain unknown. The majority of published cases were sporadic. We report on the natural history of a 32-year-old man with de novo Möbius syndrome. The diagnosis was established at the age of 9 months due to partial bilateral facial and abducent nerve palsy. Additionally, the patient demonstrated failure to thrive during infancy and childhood, many dysmorphic features, lower limb anomalies, and hypogonadism in adulthood, but his intelligence was in the normal range. The low quality of life of the patient with Möbius syndrome is emphasized.

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Section: Mo or Rp Ph Ho Ol Lo Og Gi Ic Ca Al L P Ph He En No Ot Ty supporting

confidence: 92%

The natural history of Möbius syndrome in a 32-year-old man

Chorąży

Leśniewicz

Posmyk³

et al. 2011

Neurologia i Neurochirurgia Polska

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“…The association of hearing loss in patients with congenital facial nerve palsy is not clear and has not been deeply explored. It has been reported that hearing loss may be conductive or sensorineural in these patients [23]. Al Mazrou et al [24] reported on eight cases of congenital facial nerve anomaly with hearing loss.…”

Section: Discussionmentioning

confidence: 95%

Congenital bilateral facial nerve hypoplasia with sensorineural hearing loss: A case report

Hamizan¹,

Kew²,

Abdullah³

2012

International Journal of Pediatric Otorhinolaryngology

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“…[7] No diagnostic laboratory studies yield findings specific to Moebius sequence. Computed tomography (CT) or MRI may demonstrate cranial nerve nuclei hypoplasia or calcification and sometimes cerebral malformation.…”

Section: Discussionmentioning

confidence: 99%

A rare case of Moebius sequence

Kulkarni

Madhavi

Nagasudha

et al. 2012

Indian J Ophthalmol

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We report a case of an 18-year-old male who presented with watering and inability to close the left eye completely since 6 months and inability to move both eyes outward and to close the mouth since childhood. Ocular, facial, and systemic examination revealed that the patient had bilateral complete lateral rectus and bilateral incomplete medial rectus palsy, left-sided facial nerve paralysis, thickening of lower lip and inability to close the mouth, along with other common musculoskeletal abnormalities. This is a typical presentation of Moebius syndrome which is a very rare congenital neurological disorder characterized by bilateral facial and abducens nerve paralysis. This patient had bilateral incomplete medial rectus palsy which is suggestive of the presence of horizontal gaze palsy or occulomotor nerve involvement as a component of Moebius sequence.

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Audiologic results in patients with Moebiüs sequence

Cited by 6 publications

References 22 publications

The natural history of Möbius syndrome in a 32-year-old man

The natural history of Möbius syndrome in a 32-year-old man

Congenital bilateral facial nerve hypoplasia with sensorineural hearing loss: A case report

A rare case of Moebius sequence

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