Question mark ears, temporo-mandibular joint malformation and hypotonia: auriculo-condylar syndrome or a distinct entity?

We report a boy with prominent, peculiarly malformed ears, abnormality of t he ramus of the mandible and hypotonia. An isolated peculiar bilateral ear deformity named 'question mark ear' has been delineated in plastic reconstr uction surgery reviews [Cosman et al., 1970 Plast Reconstr Surg 46:454-457; Cosman (1984) Plast Reconstr Surg 73:572-576; Takato ct al. (1989) Ann Pla st Surg 22:69-73; Brodovsky (1997) Plast Reconstr Surg 100:1254-1257; Park (1998) Plast Reconstr Surg 101:1620-1623; Al-Quattan (1998) Plast Reconstr Surg 102:439-441] and a similar deformity of the ear and changes in the tem poro-mandibular joint and condyle has been described by Jampol et al. [(199 8) Am J Med Genet 75:449-452] and by Guion-Almeida et al. [(1999) Am J Med Genet 86:130-133]. The present case may be the third description of this ma lformation complex with additional clinical features characterized by hypot onia and mild developmental delay, or possibly a new distinct entity. Clin Dysmorphol 9: 277-280 (C) 2000 Lippincott Williams & Wilkins.