X-linked dystonia-parkinsonism (XDP) is a rare, adult-onset, progressive, hereditary neurological movement disorder primarily affecting Filipino men with maternal families from Panay province of the Philippines. Medical treatment modalities currently being used have offered temporary symptomatic relief. Surgical management in the form of bilateral globus pallidi internae (Gpi) deep brain stimulation (DBS) has shown promising results and is increasingly being performed in advanced centers, as reported in international literature. Presented herein is the local experience of seven (7) retrospectively reviewed cases from February 2018 to February 2019 in a tertiary center in the Philippines with a particular focus on anesthetic management. All patients were male, from Panay, and presented with progressive dystonia and parkinsonism. All patients underwent planned bilateral, simultaneous DBS electrode, and implantable pulse generator (IPG) placement performed by a multidisciplinary team. Anesthetic management consisted of Bispectral Index (BIS) guided conscious sedation with low dose propofol and remifentanil infusions with a complete scalp nerve block (SB) at the start of the procedure then shifted to awake monitored anesthesia care during electrode placement, microelectrode recording (MER) and macro stimulation testing. All were put under general anesthesia with a supraglottic airway device during the placement of the internal pulse generator (IPG) in the infraclavicular area. All seven patients had successful localization, and insertion of the DBS electrode and discharged improved. The anesthetic management of the DBS used in these cases warrants further investigation and may lead to standardization of future practice.
Deep Brain Stimulation

OBJECTIVES: To  report  a  case  of  congenital  oval  window  aplasia  (COWA)  in  a  Filipino  adult presenting   with      unilateral   maximal   conductive   hearing   loss   and   discuss   the   diagnostic considerations, pathophysiology and management.
METHODS:
Design: Case Report
Setting: Tertiary Public Referral Center
Patient: One
RESULTS: Audiometric evaluation showed a maximal unilateral left conductive hearing loss. High resolution temporal bone CT showed absence of the oval window on the left along with facial and stapes  abnormalities.    Exploratory  tympanotomy  showed  an  aberrant  facial  nerve,  monopodal and  abnormally  located  stapes  and  absent  oval  window. Postoperative  hearing  gain  achieved after a neo-oval window and Schuknecht piston wire prosthesis remained stable over two years.
CONCLUSION: A congenital minor ear anomaly classified as Cremers Class 4a in which a congenital oval  window  aplasia  was  associated  with  an  aberrant  facial  nerve  anomaly  and  a  monopodal stapes is reported. Recent literature supported the view that congenital oval window aplasia can in selected cases be amenable to various surgical approaches and a stable postoperative hearing gain is achievable in the long term.

Human ; Male ; Female ; Adult ; Ear, Middle ; Hearing