Idiopathic cervical fibrosis--a new member of IgG4-related sclerosing diseases: report of 4 cases, 1 complicated by composite lymphoma.

Idiopathic cervical fibrosis is a rare tumefactive inflammatory-sclerosing lesion involving the soft tissues of the head and neck, and a proportion of patients also have synchronous or metachronous inflammatory fibrosclerosing lesions in other anatomic sites. The latter finding suggests that this entity may represent a member of IgG4-related sclerosing diseases. We report 4 cases to support this postulation. The patients were male adults aged 42 to 89 years, who presented with an infiltrative, firm cervical mass. Two patients also had IgG4-related chronic sclerosing sialadenitis of submandibular gland and lymphadenopathy. Histologically, the cervical soft tissue lesions had ill-defined borders, consisting of coalescent nodular lymphoid aggregates accompanied by a sclerotic stroma. Nerve infiltration, skeletal muscle invasion, and phlebitis were present. There was a significant increase in IgG4 plasma cells (87 to 327 per high-power field, with IgG4/IgG ratio of 63% to 98%). In the soft tissue lesion of 1 patient, there were expansile foci comprising dense sheets of plasma cells and small lymphoid cells that exhibited κ light chain restriction and clonal immunoglobulin gene rearrangement, consistent with supervening extranodal marginal zone lymphoma. The adjacent lymph node from the same patient showed Epstein-Barr virus (EBV)-positive classical Hodgkin lymphoma with typical morphology and immunophenotype (CD30, CD15, PAX5). Thus lymphoma can supervene in the chronic inflammatory background similar to that recently documented for IgG4-related sclerosing disease of the ocular adnexa.

The development of Cantonese Lexical Neighborhood Test: a pilot study.

OBJECTIVE: This study aimed at developing a theoretically driven open-set speech recognition test for pediatric clinical population of cochlear implant and/or hearing aid users, with Cantonese Chinese as their first language, to track progress in speech recognition performance as an outcome measurement of their rehabilitation. METHODS: Six monosyllabic and six disyllabic word lists were generated from the Cantonese CHILDES language database, constructed according to the Neighborhood Activation Model. There were three lexically "easy" and three lexically "hard" word lists in each sub-test, with 25 items in each list. Four pediatric cochlear implant users and 10 hearing aid users, with bilateral congenital severe to profound sensorineural hearing impairment and below the age of 10, participated in the study. Their performances on word recognition and phoneme recognition with the new test lists, as well as the inter-list equivalency, inter-rater reliability, and face validity of the new materials, were investigated. RESULTS: Word recognition was higher among disyllables than monosyllables. Lexically "easy" disyllabic words were better recognized than their "hard" counterparts and the monosyllables. No significant difference was noted among the three lists in each combination of syllable structure and lexical property. High inter-rater reliability, as well as high correlation between Cantonese LNT score and a receptive vocabulary test score, were revealed. CONCLUSIONS: These newly developed test lists provided reliable information on spoken word recognition of pediatric hearing prosthesis users with severe to profound hearing impairment. Inter-list equivalency and inter-rater reliability allowed monitoring of rehabilitation progress on such specific pediatric clinical population with this new test. (255).