++
Polymalformative syndrome characterized by
microcephaly, typical facial features, deafness, and mental retardation.
++
Microcephaly-Deafness Syndrome.
++
Exact incidence unknown; one
case report of an affected mother and son. Suggested autosomal dominant
inheritance.
++
Microcephaly, which apparently resolved in the mother by age
26 years. Facial asymmetry with prominent glabella,
protruding lower lip, micrognathia, and low-set, cup-shaped ears were described. Both
subjects suffered from mental retardation and deafness.
++
Primary concern is possible difficult
airway management because of micrognathia. Patients with developmental delay
may be uncooperative and be better off with sedative premedication.
Kawashima H, Tsuji N: Syndrome of microcephaly, deafness/ malformed ears,
mental retardation and peculiar facies in a mother and son.
Clin Genet 31:303,
1987.
[PubMed: 3608216]