A 6‐year‐old girl with hemangioma with hearing loss in right ear

A 6-year-old girl presented to our clinic due to hemangioma on her right face since birth with hearing loss in her right ear. She presented to dental clinic due to enamel hypoplasia and caries 2 days ago. Personal history: 37-week gestation with cesarean section, the smaller of twins, birth weight 2520 g, and increase in the right extracerebellar space found at 24 weeks of pregnancy. Physical examination showed telangiectasia on right side of her face, forehead, and nasal root (Figure 1). Erythema was visible on the right upper lip (Figure 2), right maxillary gingiva, and right upper jaw. The right side of the face was slightly larger than the opposite side. On enhanced CT, there were localized bony defects at the base of the middle cranial fossa on the right side of the skull (Figure 3), while the other part of skull was intact and the brain structure was normal. Ultrasound revealed that the right part of the teeth had a little thick periodontal membrane, the gingival blood supply increased, the UCG did not show obvious abnormality, and the hearing results showed severe conductive deafness in the right ear and no obvious abnormality in the fundus of both eyes. Due to the large segmental facial hemangioma in this child, PHACE syndrome needs to be excluded, which affected about 1/3 of patients in cranial facial segmental hemangioma. PHACE (OMIM: 606519) describes a spectrum of anomalies associated with large facial infantile hemangiomas, including posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. According to the diagnostic criteria of PHACE, this patient did not meet the diagnosis of definite PHACE (segmental hemangioma or hemangioma 5 cm on the face or scalp plus 1 major criterion or 2 minor criteria) or possible PHACE (hemangioma 5 cm on the face or scalp plus 1 minor criterion),1 so we diagnosed this patient as segmental hemangioma, cranial vascular anomaly, bony defects of the skull, conductive deafness (ossicular deformity), and enamel hypoplasia. Although this patient did not meet the criteria of PHACE, the hearing loss was related to the congenital structural abnormalities. In PHACE syndrome, the association of hearing loss has been reported in the literature,2 and 16.7% (1/6) of them had isolated conductive hearing loss.3 We proposed that our patient's hearing loss was associated with the ossicular deformity. According to the erythema on the dental and the increased blood supply under the ultrasound, the enamel hypoplasia was related to the hemangioma. In the follow-up of PHACE, 67% reported dental issues, and 58% reported enamel problems,2 so in our patient, we proposed the enamel hypoplasia was related to the hemangioma. Therefore, large segmental hemangioma may be associated with congenital dysplasia. When treating children with enamel dysplasia or ossicular dysplasia as the main complaint in the clinic, we should pay attention to asking the history of facial hemangioma. There was no indication of surgical intervention for cranial vascular malformation and hearing abnormality. This article reveals a rare case that inspires us to check for ossicular deformity when a facial hemangioma is associated with hearing loss. This work was supported by the General Project of Natural Science Foundation of Inner Mongolia Autonomous Region (2020MS08112). The authors have no conflicts of interest to declare. Written informed consent was obtained from the patient's for the publication of this report and accompanying images. Not applicable.